Soft Tissue Sarcomas
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Soft tissue sarcomas are malignant tumours that may arise in any of the mesodermal tissues (muscles, tendons, vessels that carry blood or lymph, joints, and fat). Sarcomas are a diverse range of tumours, they are named after the type of soft tissue cell they arise from. Types of soft tissue sarcomas include; alveolar soft-part sarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma, hemangiopericytoma, mesenchymoma, schwannoma, peripheral neuroectodermal tumours, rhabdomyosarcoma, synovial sarcoma, and other types. In terms of treatment these different sub-types are usually treated in the same way using a uniform soft tissue protocol.

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Information for Patients and the Public
Information for Health Professionals / Researchers
Latest Research Publications
Childhood Soft Tissue Sarcoma
Uterine Sarcoma
Kaposi Sarcoma
Gastrointestinal Stromal Tumors

Information Patients and the Public (16 links)

Information for Health Professionals / Researchers (19 links)

Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Gbabe OF, Okwundu CI, Dedicoat M, Freeman EE
Treatment of severe or progressive Kaposi's sarcoma in HIV-infected adults.
Cochrane Database Syst Rev. 2014; 8:CD003256 [PubMed] Article available free on PMC after 13/08/2015 Related Publications
BACKGROUND: Kaposi's sarcoma remains the most common cancer in Sub-Saharan Africa and the second most common cancer in HIV-infected patients worldwide. Since the introduction of highly active antiretroviral therapy (HAART), there has been a decline in its incidence.However, Kaposi's sarcoma continues to be diagnosed in HIV-infected patients.
OBJECTIVES: To assess the added advantage of chemotherapy plus HAART compared to HAART alone; and the advantages of different chemotherapy regimens in HAART and HAART naive HIV infected adults with severe or progressive Kaposi's sarcoma.
SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE and , GATEWAY, the WHO Clinical Trials Registry Platform and the US National Institutes of Health's for ongoing trials and the Aegis archive of HIV/AIDS for conference abstracts. An updated search was conducted in July 2014.
SELECTION CRITERIA: Randomised trials and observational studies evaluating the effects of any chemotherapeutic regimen in combination with HAART compared to HAART alone, chemotherapy versus HAART, and comparisons between different chemotherapy regimens.
DATA COLLECTION AND ANALYSIS: Two review authors assessed the studies independently and extracted outcome data.We used the risk ratio (RR) with a 95% confidence interval (CI) as the measure of effect.We did not conduct meta-analysis as none of the included trials assessed identical chemotherapy regimens.
MAIN RESULTS: We included six randomised trials and three observational studies involving 792 HIV-infected adults with severe Kaposi's sarcoma.Seven studies included patients with a mix of mild to moderate (T0) and severe (T1) Kaposi's sarcoma. However, this review was restricted to the subset of participants with severe Kaposi's sarcoma disease.Studies comparing HAART plus chemotherapy to HAART alone showed the following: one trial comparing HAART plus doxorubicin,bleomycin and vincristine (ABV) to HAART alone showed a significant reduction in disease progression in the HAART plus ABV group (RR 0.10; 95% CI 0.01 to 0.75, 100 participants); there was no statistically significant reduction in mortality and no difference in adverse events. A cohort study comparing liposomal anthracyclines plus HAART to HAART alone showed a non-statistically significant reduction in Kaposi's sarcoma immune reconstitution inflammatory syndrome in patients that received HAART plus liposomal anthracyclines (RR 0.49; 95% CI 0.16 to 1.55, 129 participants).Studies comparing HAART plus chemotherapy to HAART plus a different chemotherapy regimen showed the following: one trial involving 49 participants and comparing paclitaxel versus pegylated liposomal doxorubicin in patients on HAART showed no difference in disease progression. Another trial involving 46 patients and comparing pegylated liposomal doxorubicin versus liposomal daunorubicin showed no participants with progressive Kaposi's sarcoma disease in either group.Studies comparing different chemotherapy regimens in patients from the pre-HAART era showed the following: in the single RCT comparing liposomal daunorubicin to ABV, there was no significant difference with the use of liposomal daunorubicin compared to ABV in disease progression (RR 0.78; 95% CI 0.34 to 1.82, 227 participants) and overall response rate. Another trial involving 178 participants and comparing oral etoposide versus ABV demonstrated no difference in mortality in either group. A non-randomised trial comparing bleomycin alone to ABV demonstrated a higher median survival time in the ABV group; there was also a non-statistically significant reduction in adverse events and disease progression in the ABV group (RR 11; 95% CI 0.67 to 179.29, 24 participants).An additional non-randomised study showed a non-statistically significant overall mortality benefit from liposomal doxorubicin as compared to conservative management consisting of either bleomycin plus vinblastine, vincristine or single-agent antiretroviral therapy alone (RR 0.93; 95% CI 0.75 to 1.15, 29 participants). The overall quality of evidence can be described as moderate quality. The quality of evidence was downgraded due to the small size of many of the included studies and small number of events.
AUTHORS' CONCLUSIONS: The findings from this review suggest that HAART plus chemotherapy may be beneficial in reducing disease progression compared to HAART alone in patients with severe or progressive Kaposi's sarcoma. For patients on HAART, when choosing from different chemotherapy regimens, there was no observed difference between liposomal doxorubicin, liposomal daunorubicin and paclitaxel.

Related: Bleomycin Doxorubicin Etoposide Kaposi Sarcoma Skin Cancer Vincristine

Panda KG, Hale MJ, Kruger D, Luvhengo TE
Comparison between preoperative biopsy and post-excision histology results in sarcoma: experience at Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa.
S Afr J Surg. 2014; 52(2):45-8 [PubMed] Related Publications
BACKGROUND: Tumour size, grade and subtype are the main prognostic factors in adult patients presenting with soft-tissue sarcoma. Planning for appropriate management, including the need for additional staging investigations and neoadjuvant therapy, is dependent on reliable preoperative histopathological results.
OBJECTIVES: To determine whether there is agreement between preoperative and post-excision histological findings in patients presenting with soft-tissue sarcoma, and whether the agreement is influenced by the subtypes of sarcomas.
PATIENTS AND METHODS: Records of adult patients who had soft-tissue sarcomas excised were reviewed. Kaposi's sarcoma and gastrointestinal stromal tumours were excluded. Data were retrieved from the Department of Anatomical Pathology of the National Health Laboratory Service and theatre records at Chris Hani Baragwanath Academic Hospital, and included patient demography, tumour sites and size, HIV status, biopsy types and post-excision histological findings.
RESULTS: Records of 153 patients were found (median age 44 years). The majority of the sarcomas were >5 cm in diameter, deep seated and localised in extremities. The commonest subtype, irrespective of HIV status, was dermatofibrosarcoma protuberans. Fine-needle aspiration biopsy (FNAB) results were inaccurate in determining the malignant nature, grade and subtype of sarcoma. Rates of accurate tumour subtype classification following core needle and incision biopsies when compared with post-excision histological findings were 73.1% and 78.3%, respectively.
CONCLUSION: FNAB should not be used in the primary evaluation of soft-tissue tumours. A report of spindle cells on the FNAB smear should be followed by core needle or incision biopsy. Incision biopsy is superior to core needle biopsy in the classification of sarcomas by subtype.

Bryan ML, Fitzgerald NC, Levine EA, et al.
Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy in sarcomatosis from gastrointestinal stromal tumor.
Am Surg. 2014; 80(9):890-5 [PubMed] Article available free on PMC after 01/09/2015 Related Publications
The role of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) procedures in the management of patients with gastrointestinal stromal tumor (GIST)-induced sarcomatosis that is refractory to tyrosine kinase inhibitors (TKI) is not well defined. A retrospective analysis of a prospective database of 1070 CRS/HIPEC procedures was performed. Demographics, Eastern Cooperative Oncology Group performance status, resection status, morbidity, mortality, perioperative use of targeted therapies, and overall survival were analyzed. Since 1992, 18 CRS/HIPEC procedures were performed for peritoneal dissemination of GIST. Fifty per cent of these cases were performed before the introduction of TKIs. R0/1 resection was achieved in 72 per cent, whereas 63 per cent of patients were treated with neoadjuvant and/or adjuvant targeted therapy. Thirty-day morbidity and mortality were 33.3 and 5.6 per cent, respectively. Median overall survival after CRS/HIPEC was 3.33 years with 3-year survival of 56 per cent. Median survival in those who did not receive targeted therapy was 1.04 versus 7.9 years for those treated with TKI and cytoreduction. Median postsurgical survival for those treated preoperatively with progression on TKI treatment was 1.35 years versus not reached in those on TKI therapy without progression. Primary therapy for patients with disseminated GIST should be TKI therapy. However, in patients with sarcomatosis from GIST, cytoreduction should be considered before developing TKI resistance. Progression on TKI is associated with poor outcomes even after complete cytoreduction.

Related: Gastrointestinal System Cancers Gastrointestinal Stromal Tumors

Sole CV, Calvo FA, Polo A, et al.
Anticipated intraoperative electron beam boost, external beam radiation therapy, and limb-sparing surgical resection for patients with pediatric soft-tissue sarcomas of the extremity: a multicentric pooled analysis of long-term outcomes.
Int J Radiat Oncol Biol Phys. 2014; 90(1):172-80 [PubMed] Related Publications
PURPOSE: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas.
METHODS AND MATERIALS: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis.
RESULTS: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3).
CONCLUSIONS: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results.

Related: Synovial Sarcoma

Bhalla A, Sandhu F, Sieber S
Primary adrenal leiomyosarcoma: a case report and review of the literature.
Conn Med. 2014; 78(7):403-7 [PubMed] Related Publications
Primary adrenal leiomyosarcoma has been reported previously in 25 patients. The patient presented herein is the only case where the definitive diagnosis was made with core needle biopsy evaluation. A 45-year-old male presented with pain in the back and right groin. Radiological evaluation demonstrated a heterogeneous 11 cm right adrenal mass, multiple liver masses, and an enlarged aortocaval lymph node. No retroperitoneal mass was identified. Core needle biopsies revealed a malignant mesenchymal neoplasm composed of atypical spindle shaped cells arranged in intersecting fascicles, with high mitotic activity and focal tumor necrosis. Immunohistochemical stains revealed immunoreactivity for smooth muscle actin and desmin. S-100 and c-kit stains were negative. The diagnosis of adrenal leiomyosarcoma with liver metastasis was rendered. It was an aggressive tumor with clinical presentation at Figure 1. Core needle biopsy, a an advanced stage. Definitive diagnosis of this tumor by core needle biopsy can obviate the need for surgical biopsy in patients with advanced disease.

Kara HV, Javidfar J, D'Amico TA
Surgical excision for mediastinal synovial sarcoma with limited response to chemoradiotherapy.
Ann Thorac Surg. 2014; 98(3):e69-70 [PubMed] Related Publications
Primary synovial sarcoma of the mediastinum is an exceedingly rare neoplasm. We describe a 31-year-old woman who had an incidental diagnosis of mediastinal mass. Histopathology and immunohistochemistry analysis confirmed the diagnosis of primary mediastinal synovial sarcoma. The patient underwent concurrent chemotherapy and radiotherapy, with minimal response radiologically. Resection was subsequently performed, with negative margins. The histopathologic examination revealed the diagnosis with a limited pathologic response. Because of the rarity of primary mediastinal synovial sarcoma, the optimal therapy is still unclear. We report this case of induction therapy followed by en bloc surgical resection.

Related: Synovial Sarcoma

Hata A, Sekine Y, Koh E, Hiroshima K
Operative wound implantation of inflammatory sarcomatoid carcinoma of the lung.
Ann Thorac Surg. 2014; 98(3):1111-3 [PubMed] Related Publications
We describe a patient with iatrogenic chest wall implantation of inflammatory sarcomatoid carcinoma. A 43-year-old man underwent right partial lung resection for hemopneumothorax, with large bullae and an alveolar accumulation of histiocytes found on pathology. Three months later, a subcutaneous tumor appeared at a thoracoscopic port site. Needle aspiration of this tumor suggested a malignant neoplasm; therefore, a right upper lobectomy and chest wall resection were performed, and a pathologic diagnosis of sarcomatoid carcinoma was made. Pathologic reexamination of the original sample suggested that the tumor has been implanted in the patient's chest wall at the time of the first operation.

Related: Lung Cancer

Reuter S, Vrachimis A, Huss S, et al.
A challenging case of rapid progressive Kaposi sarcoma after renal transplantation: diagnostics by FDG PET/CT.
Medicine (Baltimore). 2014; 93(11):e67 [PubMed] Related Publications
De-novo malignancy is a serious posttransplant complication. While the incidence of Kaposi sarcoma (KS) is low, the time for its diagnosis is early after renal transplantation. Typically, it can be identified because of the classical skin lesion. We herein report an unusual case of rapid progressive KS without skin lesions in a 52-year-old patient leading to death within 8 months after kidney transplantation. This striking case illustrates the usefulness of [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography for demonstrating the cause of unexplained deterioration of patient's condition. Early identification of KS is critical because early (modification of) therapy can substantially improve patient's prognosis.

Related: Kaposi Sarcoma

Schuh R, Panotopoulos J, Puchner SE, et al.
Vascularised or non-vascularised autologous fibular grafting for the reconstruction of a diaphyseal bone defect after resection of a musculoskeletal tumour.
Bone Joint J. 2014; 96-B(9):1258-63 [PubMed] Related Publications
Resection of a primary sarcoma of the diaphysis of a long bone creates a large defect. The biological options for reconstruction include the use of a vascularised and non-vascularised fibular autograft. The purpose of the present study was to compare these methods of reconstruction. Between 1985 and 2007, 53 patients (26 male and 27 female) underwent biological reconstruction of a diaphyseal defect after resection of a primary sarcoma. Their mean age was 20.7 years (3.6 to 62.4). Of these, 26 (49 %) had a vascularised and 27 (51 %) a non-vascularised fibular autograft. Either method could have been used for any patient in the study. The mean follow-up was 52 months (12 to 259). Oncological, surgical and functional outcome were evaluated. Kaplan-Meier analysis was performed for graft survival with major complication as the end point. At final follow-up, eight patients had died of disease. Primary union was achieved in 40 patients (75%); 22 (42%) with a vascularised fibular autograft and 18 (34%) a non-vascularised (p = 0.167). A total of 32 patients (60%) required revision surgery. Kaplan-Meier analysis revealed a mean survival without complication of 36 months (0.06 to 107.3, sd 9) for the vascularised group and 88 months (0.33 to 163.9, sd 16) for the non-vascularised group (p = 0.035). Both groups seem to be reliable biological methods of reconstructing a diaphyseal bone defect. Vascularised autografts require more revisions mainly due to problems with wound healing in distal sites of tumour, such as the foot.

Related: Bone Cancers

Zemanova M, Rauova K, Boljesikova E, et al.
Analysis of radiation-induced angiosarcoma of the breast.
Bratisl Lek Listy. 2014; 115(5):307-10 [PubMed] Related Publications
Breast angiosarcoma may occur de novo, or as a complication of radiation therapy, or chronic lymphedema secondary to axillary lymph node dissection for mammary carcinoma. Both primary and secondary angiosarcomas may present with bruise like skin discoloration, which may delay the diagnosis. Imaging findings are nonspecific. In case of high-grade tumours, MRI may be used effectively to determine lesion extent by showing rapid enhancement, nevertheless earliest possible diagnostics is crucial therefore any symptoms of angiosarcoma have to be carefully analysed. The case analysed here reports on results of 44-year old premenopausal woman who was treated for a T1N1M0 invasive ductal carcinoma. After a biopsy diagnosis of carcinoma, the patient underwent quadrantectomy with axillary lymph node dissection. She received partial 4 cycles of chemotherapy with adriamycin and cyclophosphamide, followed by radiation treatment. Thereafter, a standard postoperative radiotherapy was applied at our institution four months after chemotherapy (TD 46 Gy in 23 fractions followed by a 10 Gy electron boost to the tumour bed). Adjuvant chemotherapy was finished six months after operation, followed by tamoxifen. Follow up: no further complications were detected during regular check-ups. However, 12-years later, patient reported significant changes at breast region which was exposed to radiation during treatment of original tumour. In this article, we describe the clinical presentation, imaging and pathological findings of secondary angiosarcoma of the breast after radiotherapy (Fig. 2, Ref. 26).

Related: Breast Cancer

Tang YJ, Wang JL, Nong LG, et al.
Associations of IL-27 polymorphisms and serum IL-27p28 levels with osteosarcoma risk.
Medicine (Baltimore). 2014; 93(10):e56 [PubMed] Related Publications
Interleukin (IL)-27 is a novel cytokine secreted by stimulation of antigen-presenting cells. No previous studies currently reported the role of IL-27 in the carcinogenesis of osteosarcoma. We aimed to investigate the association of IL-27 polymorphisms and serum IL-27p28 with osteosarcoma risk in a Chinese population.One hundred and sixty osteosarcoma patients and 250 health controls were selected. IL-27 gene -964 A/G, 2905 T/G, and 4730 T/C polymorphisms were determined by using polymerase chain reaction-restriction fragment length polymorphism. Enzyme-linked immunosorbent assay were used to detect serum IL-27p28 levels.The serum IL-27p28 levels were significantly lower in osteosarcoma patients compared with those in controls (P < 0.01). Serum IL-27p28 levels in stages III-IV were lower than those in stages I-II of osteosarcoma (P < 0.05); similar results were also found in patients with metastasis, that is, patients with metastasis have higher IL-27p28 levels than those without metastasis (P < 0.05). There were no associations between genotype and allele frequencies of IL-27 -964 A/G, 2905 T/G, 4730 T/C, and the risk of osteosarcoma (P > 0.05). Stratification analysis also failed to show the associations between -964 A/G, 2905 T/G, and 4730 T/C polymorphisms and the clinical stage and metastasis of osteosarcoma (P > 0.05). Three possible haplotypes (ATT, GTT, and GGC) were identified, but no associations were found between them and the osteosarcoma risk (P > 0.05).This study indicates that the lower serum IL-27p28 levels may be associated with development and progression of osteosarcoma, but IL-27 gene -964 A/G, 2905 T/G, and 4730 T/C polymorphisms and their haplotypes are not associated with osteosarcoma risk.

Related: Bone Cancers Osteosarcoma

Musser JE, Assel M, Mashni JW, et al.
Adult prostate sarcoma: the Memorial Sloan Kettering experience.
Urology. 2014; 84(3):624-8 [PubMed] Related Publications
OBJECTIVE: To present our institutional experience with adult prostate sarcoma over 30 years.
MATERIALS AND METHODS: We reviewed 38 cases of adult prostate sarcoma diagnosed and treated at our institution between 1982 and 2012. Univariate Cox proportional hazards regression was used to determine if there was an association between specific disease characteristics (tumor size, histology, American Joint Committee on Cancer stage, and metastasis at diagnosis) and cancer-specific survival (CSS).
RESULTS: A total of 38 patients were included, with a median age of 50 years (range, 17-73 years). Most men presented with lower urinary tract symptoms (45%), hematuria (24%), or acute urinary retention (21%). Diagnosis was established with prostate needle biopsy (68%) or transurethral resection of the prostate (18%). The predominant histologic subtypes were leiomyosarcoma (13 cases, 34%) and rhabdomyosarcoma (12 cases, 32%). Rhabdomyosarcoma was associated with poorer CSS (hazard ratio, 3.00; 95% confidence interval [CI], 1.13-7.92; P = .027) compared with leiomyosarcoma. We did not observe a significant relationship between tumor size and CSS. Overall, median CSS was 2.9 years (95% CI, 1.5-5.4), with 7.7 years for clinically localized disease (95% CI 2.5; upper bound not reached) and 1.5 years for metastatic disease (95% CI 1.1, 2.7).
CONCLUSION: Adult prostate sarcoma has a poor prognosis, especially in cases of metastatic disease at the time of diagnosis. Surgery remains the standard of care, but it provides limited benefit to those with metastatic disease or as a consolidation therapy after partial response to systemic therapy.

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Xiao A, Li Z, He X, You C
A rare tentorial mesenchymal chondrosarcoma in posterior cranial fossa: case report.
Neurol Neurochir Pol. 2014; 48(4):287-91 [PubMed] Related Publications
Intracranial extraskeletal mesenchymal chondrosarcoma is a very rare malignant tumor with predilection site of frontoparietal falx cerebri. Only few cases of mesenchymal chondrosarcoma in posterior cranial fossa are reported. Here, we report a 23-year-old young man with a dura-attached mass in left posterior cranial fossa misdiagnosed as a tentorial meningioma preoperatively. According to the following operation, the lesion was confirmed as mesenchymal chondrosarcoma surgically and pathologically. On MRI, the tumor was characterized by lobulated soft-tissue mass with dura-attached base, patchy calcifications and heterogeneous signal intensities. On contrast-enhanced MRI, it was well-defined, with marked enhancement. We consider that these imaging features above might remind us to consider the diagnosis of mesenchymal chondrosarcoma in posterior cranial fossa. The postoperative treatment of radiotherapy is still controversial. As for our case, according to the 24 months follow-up after postoperative γ-knife, our patient shows an optimistic prognosis so far.

Related: Mesenchymal Chondrosarcoma

Bulte JW
Science to Practice: Highly shifted proton MR imaging--a shift toward better cell tracking?
Radiology. 2014; 272(3):615-7 [PubMed] Related Publications
Summary A "hot spot" magnetic resonance (MR) imaging cell tracking technique has been developed that allows direct detection of dysprosium- or thulium-1,4,7,10-tetraazacyclododecane-α,α',α'',α'''-tetramethyl-1,4,7,10-tetraacetic acid (DOTMA)-labeled protons inside cells. These highly shifted protons may allow specific detection of multiple cell types because it does not rely on acquiring the proton signal from bulk water.

Soipi S, Vucić M, Ulamec M, et al.
Leiomyosarcoma of the spermatic cord with scalp metastasis: case report and literature review.
Coll Antropol. 2014; 38(2):763-6 [PubMed] Related Publications
Herein we present 82-year-old man with leiomyosarcoma arising from the spermatic cord with scalp metastasis, five years after primary surgical treatment. Complete surgical excision is required in such cases, as well as precise evaluation of further therapy. Paratesticular leiomyosarcoma is a rare entity, malignant mesenchimal tumor of smooth muscle differentiation. Although leiomyosarcomas of different localizations have well-known metastatic potential, cutaneous metastases are extremely rare with only 16 cases described in the literature. To our knowledge there are no reported cases of the paratesticular leiomyosarcoma metastatic to the skin. This article reviews the literature regarding paratesticular leiomyosarcoma presentation, diagnosis and treatment.

Paner GP, Cox RM, Richards K, et al.
Pseudoangiosarcomatous urothelial carcinoma of the urinary bladder.
Am J Surg Pathol. 2014; 38(9):1251-9 [PubMed] Related Publications
The pseudoangiosarcomatous pattern has been described mostly in cutaneous and some visceral squamous cell carcinomas and is unique for its striking morphologic resemblance to angiosarcoma. Herein, we describe the clinicopathologic features of 7 pseudoangiosarcomatous urothelial carcinomas that occurred in the urinary bladder. The patients included 6 men and 1 woman ranging in age from 47 to 87 years (median 70 y). The pseudoangiosarcomatous morphology was observed in 7 urothelial carcinomas including 3 with squamous differentiation and comprised 35% to 85% of the invasive tumor. Histologically, the pseudoangiosarcomatous carcinomas were characterized by tumor cell discohesion and lysis that created pseudolumina formations surrounded by attached residual tumor cells. Detached degenerating tumor cells variably admixed with inflammatory cells were common in the false lumina. Partly intact urothelial carcinoma nests contained irregular or cleft-like spaces and disintegrating tumor cells with stretched intercellular bridges. The tumor was commonly associated with a dense collagenous matrix, often surrounding the lytic nests. Similar tumor cell discohesion and breakdown were observed in 3 tumors with foci of squamous cell differentiation, distinguished by the presence of dyskeratosis and keratin formation. All 7 tumors contained other nonpseudoangiosarcomatous carcinoma components such as conventional urothelial carcinoma (5), squamous differentiation (4), sarcomatoid spindle cell carcinoma (2), small cell carcinoma (1), micropapillary carcinoma (1), and glandular differentiation (1). The pseudoangiosarcomatous urothelial carcinomas were all (7/7) diffusely CK7 positive, most (6/7) were GATA3 positive, and none (0/7) expressed vascular-associated markers. There was no evidence to suggest that apoptosis (by TUNEL assay and cleaved caspase-3 immunostaining) or loss of the adhesion molecules CD138 and e-cadherin were possible causes for the tumor cell discohesion and breakdown. All 7 tumors were high stage at cystectomy and included 1 pT3a, 2 pT3b, and 4 pT4a tumors, and 3 had pelvic lymph node involvement. Follow-up data available in 6 cases revealed a poor outcome with an overall median survival of 8.5 months. In conclusion, we present an unusual morphology of bladder carcinoma that has a striking resemblance to a malignant vasoformative tumor. Our series showed that bladder pseudoangiosarcomatous carcinoma morphology is associated with a higher tumor stage at cystectomy, commonly admixed with other aggressive carcinoma variant morphologies, and portend a poorer outcome. Knowledge of this pattern is also important to avoid misdiagnosis, particularly in limited tissue samples.

Related: Bladder Cancer Bladder Cancer - Molecular Biology

Sciallis AP, Bedroske PP, Schoolmeester JK, et al.
High-grade endometrial stromal sarcomas: a clinicopathologic study of a group of tumors with heterogenous morphologic and genetic features.
Am J Surg Pathol. 2014; 38(9):1161-72 [PubMed] Related Publications
The existence of a "high-grade endometrial stromal sarcoma" category of tumors has been a controversial subject owing to, among other things, the difficulty in establishing consistent diagnostic criteria. Currently, the recommended classification for such tumors is undifferentiated uterine/endometrial sarcoma. Interest in this subject has recently increased markedly with the identification of recurrent molecular genetic abnormalities. At Mayo Clinic, a group of neoplasms has been observed that morphologically resemble, either cytologically or architecturally, classic "low-grade" endometrial stromal sarcoma but feature obvious deviations, specifically, 17 tumors with unequivocally high-grade morphology. These high-grade tumors displayed 3 morphologic themes: (1) tumors with a component that is identical to low-grade ESS that transitions abruptly into an obviously higher-grade component; (2) tumors composed exclusively of high-grade cells with uniform nuclear features but with a permeative pattern of infiltration; (3) tumors similar to the second group but with a different, yet characteristic, cytomorphology featuring enlarged round to ovoid cells (larger than those found in low-grade ESS) with smooth nuclear membranes and distinct chromatin clearing but lacking prominent nucleoli. We collected clinicopathologic data, applied immunohistochemical studies, and also tested tumors by fluorescence in situ hybridization for abnormalities in JAZF1, PHF1, YWHAE, and CCND1. Tumors from these 3 groups were found to be immunohistochemically and genetically distinct from one another. Most notable was the fact that category 3 contained all the cases that tested positive for YWHAE rearrangement, did not show any classic translocations for JAZF1, PHF1, or CCND1, often presented at a high stage, and behaved aggressively. This study demonstrates the morphologic, immunophenotypic, and molecular genetic heterogeneity that exists within "undifferentiated endometrial sarcomas" as currently defined and lends credence to the effort of subclassifying some tumors as truly "high-grade endometrial stromal sarcomas." Our study also shows that, in the context of undifferentiated endometrial sarcomas, recognition of cytomorphologic features on routine hematoxylin and eosin-stained sections may be used to select tumors with specific molecular genetic changes-that is, translocations involving YWHAE. Our conclusions will help further efforts towards proper sub-classification of these tumors which will aid in diagnosis and potentially affect clinical management.

Related: Endometrial (Uterus) Cancer Endometrial Cancer FISH

Yasir S, Gamez R, Jenkins S, et al.
Significant histologic features differentiating cellular fibroadenoma from phyllodes tumor on core needle biopsy specimens.
Am J Clin Pathol. 2014; 142(3):362-9 [PubMed] Related Publications
OBJECTIVES: Cellular fibroepithelial lesions (CFELs) are a heterogeneous group of tumors encompassing cellular fibroadenoma (CFA) and phyllodes tumor (PT). Distinction between the two is challenging on core needle biopsy (CNB) specimens. The objective of this study was to evaluate histologic features that can help distinguish PT from CFA on CNB specimens.
METHODS: Records of all patients diagnosed with CFELs on CNB specimens with follow-up excision between January 2002 and December 2012 were retrieved. Histopathologic stromal features were evaluated on CNB specimens, including mitoses per 10 high-power fields (hpf), overgrowth, increased cellularity, fragmentation, adipose tissue infiltration, heterogeneity, subepithelial condensation, and nuclear pleomorphism.
RESULTS: Twenty-seven (42.2%) of 64 were diagnosed as PT (24 benign PTs and three borderline PTs) and 37 (57.8%) as CFA on excision. All features except for increased stromal cellularity were statistically significant. The average number of histologic features seen in PT and CFA was 3.9 and 1.4, respectively (odds ratio [OR], 7.27; 95% confidence interval [CI], 2.44-21.69; P = .0004). The average number of mitoses per 10 hpf was 3.0 for PT compared with 0.8 for CFA (OR, 2.14; 95% CI, 1.18-3.86; P = .01).
CONCLUSIONS: The presence of mitoses (three or more) and/or total histologic features of three or more on CNB specimens were the most helpful features in predicting PT on excision.

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Miyata H, Tsuji N, Jimi T, et al.
Adenosarcoma of the uterine body initially presenting as an interstitial small tumor of the uterus: a case report.
Eur J Gynaecol Oncol. 2014; 35(4):473-6 [PubMed] Related Publications
Adenosarcoma of the uterine body is a rare mixed tumor in which a benign epithelial component is mixed with a malignant stromal element. It has been considered that this tumor originates from the endometrium and its most common finding of imaging is a polypoid tumor occupying the uterine cavity. The authors herein present a case of 37-year-old female with a complaint of abnormal vaginal bleeding. At the first visit, transvaginal ultrasound and magnetic resonance imaging (MRI) showed a round mass with a diameter of one cm in the uterine wall. No malignant pathological finding was detected. The patient visited the authors again one year later, because of continuous bleeding. At that time, they found a polypoid tumor in the uterine cavity, which turned out to be adenosarcoma with sarcomatous overgrowth. The round mass in the uterus detected at first time seems to have been incipience of adenosarcoma. Prodromal sign of adenosarcoma has not been reported previously.

Grigore M, Ilea C, Terinte C, et al.
Heterologous type of malignant mixed Müllerian tumor of the uterus presenting as a vulvar mass.
Eur J Gynaecol Oncol. 2014; 35(4):458-60 [PubMed] Related Publications
Carcinosarcoma is a rare, extremely aggressive tumor of the uterus with a poor prognosis. The authors describe a case of a 78-year-old woman who presented with a giant mass protruding through the cervix, vagina, and vulva. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. The histopathological examination of the surgical specimen revealed a malignant mixed Müllerian tumor. The clinical and pathological features, molecular data, and prognosis of this aggressive neoplasm are discussed. Although uterine carcinosarcomas are extremely rare, when a postmenopausal woman with a vulvar mass is admitted to the gynecology clinic, the physician should consider that the mass may be a carcinosarcoma.

Yoon BS, Seong SJ, Song T, et al.
Rectus abdominis muscle resection and fascial reconstruction for the treatment of uterine leiomyosarcoma invading the abdominal wall: a case report.
Eur J Gynaecol Oncol. 2014; 35(4):449-51 [PubMed] Related Publications
The authors present a case of intra-abdominal recurrent leiomyosarcoma invading a large area of the abdominal wall. The patient underwent cytoreductive surgery, including resection of the rectus abdominis muscle, followed by reconstruction of the defect using synthetic mesh. The tumor was surgically removed by en bloc resection, including most of the rectus abdominis muscle and ileum. The abdominal wall defect was repaired using synthetic mesh. The patient underwent radiotherapy and chemotherapy after the surgery and was healthy one year later.

Wang XY, Kang LK, Lan CY
Contrast-enhanced ultrasonography in diagnosis of benign and malignant breast lesions.
Eur J Gynaecol Oncol. 2014; 35(4):415-20 [PubMed] Related Publications
PURPOSE: This study aims to investigate the value of real-time contrast-enhanced ultrasonography (US) in differentiating benign and malignant breast lumps.
MATERIALS AND METHODS: Patients with breast lesions were observed. The enhancement form, intensity, and time-intensity curve were classified, and the characteristics of all the lesions were analyzed.
RESULTS: Inhomogeneous partial enhancement and entire enhancement were exhibited in most of the malignant lesions. High enhancements were observed in malignant lesions, whereas lower enhancement and no enhancement were exhibited in the benign tumors. The peak value and regression time were significantly different between the two groups.
CONCLUSION: There's a significant difference regarding the results of real-time contrast-enhanced ultrasound between the benign tumor and malignant tumor which would help to improve the diagnostic accuracy of breast neoplasms.

Related: Breast Cancer

Svalina MN, Keller C
YAPping about differentiation therapy in muscle cancer.
Cancer Cell. 2014; 26(2):154-5 [PubMed] Article available free on PMC after 11/08/2015 Related Publications
Overcoming a presumed differentiation block in the childhood muscle cancer embryonal rhabdomyosarcoma is often thought to hold promise as an approach to replace cytotoxic chemotherapy with molecularly-targeted differentiation therapies. In this issue of Cancer Cell, Tremblay and colleagues implicate YAP1 and the Hippo signaling pathway in the maintenance of differentiation-arrested and proliferative phenotypes for embryonal rhabdomyosarcoma.

Pantazis D, Liappi G, Riga M
Chondrosarcoma treated with transoral near total resection of the cricoid.
B-ENT. 2014; 10(2):121-5 [PubMed] Related Publications
INTRODUCTION: Chondrosarcomas of the larynx are usually slow-growing tumours. Their prognosis is reportedly unaffected by local tumour recurrence. Nevertheless, total laryngectomy is often performed in fear that resection of the posterior lamina of the cricoid cartilage may cause laryngeal collapse and stenosis. Transoral laser surgery (TLS) is not considered among the treatment options. This case report supports the feasibility of a radical yet organ- and function-preserving tumour excision using TLS.
CASE REPORT: A female patient presented with dyspnoea due to an extensive low-grade laryngeal chondrosarcoma. TLS treatment involved total resection of the posterior lamina of the cricoid cartilage. The patient was decannulated 8 months later with normal swallowing and satisfactory voice quality, which allowed her to have a normal personal and social life.
CONCLUSION: TLS excision of the posterior lamina of the cricoid cartilage seems to be a feasible and radical yet function- and organ-preserving technique with minimal morbidity.

Related: Chondrosarcoma Cancer of the Larynx Laryngeal Cancer - Molecular Biology

Tremblay AM, Missiaglia E, Galli GG, et al.
The Hippo transducer YAP1 transforms activated satellite cells and is a potent effector of embryonal rhabdomyosarcoma formation.
Cancer Cell. 2014; 26(2):273-87 [PubMed] Related Publications
The role of the Hippo pathway effector YAP1 in soft tissue sarcomas is poorly defined. Here we report that YAP1 activity is elevated in human embryonal rhabdomyosarcoma (ERMS). In mice, sustained YAP1 hyperactivity in activated, but not quiescent, satellite cells induces ERMS with high penetrance and short latency. Via its transcriptional program with TEAD1, YAP1 directly regulates several major hallmarks of ERMS. YAP1-TEAD1 upregulate pro-proliferative and oncogenic genes and maintain the ERMS differentiation block by interfering with MYOD1 and MEF2 pro-differentiation activities. Normalization of YAP1 expression reduces tumor burden in human ERMS xenografts and allows YAP1-driven ERMS to differentiate in situ. Collectively, our results identify YAP1 as a potent ERMS oncogenic driver and a promising target for differentiation therapy.

de Chaumont A, Pierret C, de Kerangal X, et al.
Leiomyosarcoma of the superior vena cava.
Ann Thorac Surg. 2014; 98(2):725-7 [PubMed] Related Publications
Leiomyosarcoma of the superior vena cava is a very rare tumor and only a few cases have been reported, with various techniques of vascular reconstruction. We describe a new case of leiomyosarcoma of the superior vena cava in a 61-year-old woman with extension to the brachiocephalic arterial trunk. Resection and vascular reconstruction were performed using, respectively, polytetrafluoroethylene and polyethylene terephtalate vascular grafts.

Ramlawi B, Al-Jabbari O, Blau LN, et al.
Autotransplantation for the resection of complex left heart tumors.
Ann Thorac Surg. 2014; 98(3):863-8 [PubMed] Related Publications
BACKGROUND: Complete surgical resection is the optimal treatment for malignant and complex benign left heart tumors. Anatomic inaccessibility and relationship with vital cardiac structures, makes complete resection of these complex tumors with standard surgical technique suboptimal. We employ autotransplantation in these cases to allow optimal anatomic exposure for complete resection and accurate reconstruction.
METHODS: From 1998 to 2013, 35 cardiac autotransplants were done in 34 patients. Demographics, tumor histology, operative notes, hospital data, pathology reports, morbidity, and short and long-term mortality data were analyzed. Mortality follow-up was complete in all patients.
RESULTS: Of the 34 patients, there were 26 primary cardiac sarcomas, 1 isolated malignant melanoma metastasis to the intracavitary left ventricle, and 7 benign cases. The benign group had no operative deaths and 100% 2-year survival. Overall 30-day, 1-year, and 2-year procedural survival was 85%, 59%, and 44%, respectively. For primary malignant tumors, survival at 1 and 2 years was 46% and 28%. Among patients with primary malignant tumors, 19 had isolated cardiac autotransplantation and 7 had autotransplantation plus pneumonectomy. Operative mortality (and median survival) for cardiac autotransplantation with and without pneumonectomy was 43% (55 days) and 11% (378 days), respectively. For primary sarcomas, microscopically positive or negative resection margins did not impact survival.
CONCLUSIONS: Cardiac autotransplantation is a feasible and safe technique for resection of complex left-sided tumors when done as an isolated procedure in experienced centers. Addition of concomitant pneumonectomy carries a high rate of mortality and should be avoided. Further studies are needed to validate these results.

Brown MT, Gikas PD, Bhamra JS, et al.
How safe is curettage of low-grade cartilaginous neoplasms diagnosed by imaging with or without pre-operative needle biopsy?
Bone Joint J. 2014; 96-B(8):1098-105 [PubMed] Related Publications
The pre-operative differentiation between enchondroma, low-grade chondrosarcoma and high-grade chondrosarcoma remains a diagnostic challenge. We reviewed the accuracy and safety of the radiological grading of cartilaginous tumours through the assessment of, first, pre-operative radiological and post-operative histological agreement, and second the rate of recurrence in lesions confirmed as high-grade on histology. We performed a retrospective review of major long bone cartilaginous tumours managed by curettage as low grade between 2001 and 2012. A total of 53 patients with a mean age of 47.6 years (8 to 71) were included. There were 23 men and 30 women. The tumours involved the femur (n = 20), humerus (n = 18), tibia (n = 9), fibula (n = 3), radius (n = 2) and ulna (n = 1). Pre-operative diagnoses resulted from multidisciplinary consensus following radiological review alone for 35 tumours, or with the addition of pre-operative image guided needle biopsy for 18. The histologically confirmed diagnosis was enchondroma for two (3.7%), low-grade chondrosarcoma for 49 (92.6%) and high-grade chondrosarcoma for two (3.7%). Three patients with a low-grade tumour developed a local recurrence at a mean of 15 months (12 to 17) post-operatively. A single high-grade recurrence (grade II) was treated with tibial diaphyseal replacement. The overall recurrence rate was 7.5% at a mean follow-up of 4.7 years (1.2 to 12.3). Cartilaginous tumours identified as low-grade on pre-operative imaging with or without additional image-guided needle biopsy can safely be managed as low-grade without pre-operative histological diagnosis. A few tumours may demonstrate high-grade features histologically, but the rates of recurrence are not affected.

Related: Bone Cancers Chondrosarcoma

Diaconu C, Timofte D, Dănilă R, et al.
Split-thickness skin grafting in oncologic surgery of the limb--a case report.
Rev Med Chir Soc Med Nat Iasi. 2014 Apr-Jun; 118(2):423-6 [PubMed] Related Publications
Large excision of tumor soft tissues is often complicated by infections, seriously undermined blood supply and venous drainage, tendon exposure, functional impairment. We report the case of a 67 years old woman admitted for a sarcoma of the right forearm occurring 14 years after a modified radical mastectomy and adjuvant chemotherapy for carcinoma of the right breast. A wide excision of the lesion and split-thickness skin graft was performed with uneventful recovery and satisfactory healing of the graft tissue and functional aspect of the arm. In the last few decades, there has been a search for solutions in tissue repair without need for auto grafts, such as biological substitutes that could repair or improve the function of tissue. In case none of these products are available, auto graft is a good choice and it may be commonly used in the surgical treatment of postoperative soft tissues defects after oncologic surgery.

Related: Kaposi Sarcoma

Donfut AL, Lemaitre J, Van de Walle H, et al.
Primitive pulmonary "malignant epithelioid hemangioendothelioma" versus epithelioid angiosarcoma. A case report and review of the literature.
Acta Chir Belg. 2014 Mar-Apr; 114(2):143-5 [PubMed] Related Publications
We describe the case of a 56-year-old man presenting a primary pulmonary epithelioid angiosarcoma versus malignant epithelioid hemangioendothelioma still alive, without recurrence at nearly two years after the beginning of the symptoms. The primary pulmonary angiosarcoma is extremely rare, being reported only in a handful of cases. Metastatic involvement of the lung (90%) is far more common than primary pulmonary involvement (10%). Various predisposing condition for the development of angiosarcoma have been described. Early diagnosis is not common, because of the rarity of angiosarcoma in the lung and consequent low index of suspicion. Due to the paucity of cases, there are no defined treatment regimens for this entity. However, there is a tendency for surgical intervention in all reported cases.

Related: Lung Cancer

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