Vulvar sarcoma outcomes by histologic subtype: a Surveillance, Epidemiology, and End Results (SEER) database review.

OBJECTIVE: Vulvar cancers account for 5% of all gynecologic malignancies; only 1%-3% of those vulvar cancers are primary vulvar sarcomas. Given the rarity of vulvar sarcomas, outcome data specific to histopathologic subtypes are sparse. The aim of this study was to identify clinical and pathologic factors of primary vulvar sarcomas that are associated with survival and may inform treatment decisions. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was searched for women diagnosed with vulvar sarcoma between 1973 and 2018. We identified 315 patients and reviewed their demographic, clinicopathologic, surgical, and survival information. Statistical analyses included χ2 and t-tests, Kaplan-Meier survival, and Cox regression analyses. RESULTS: The most common histopathologies of vulvar sarcomas were dermatofibrosarcomas (85/315, 27%) and leiomyosarcomas (72/315, 22.9%). Rhabdomyosarcomas (18/315, 5.7%), liposarcomas (16/315, 5.1%), and malignant fibrous histiocytomas (16/315, 5.1%) were less frequent. The majority of patients underwent surgery (292/315, 92.7%), which included lymph node dissections in 21.6% (63/292). Survival and lymph node involvement varied significantly with histologic subtype. The 5-year disease-specific survival for dermatofibrosarcomas, liposarcomas, and fibrosarcomas was 100% and only 60.3% and 62.5% for malignant fibrous histiocytomas and rhabdomyosarcomas, respectively. None of the patients with (dermato)fibrosarcomas, liposarcomas, or leiomyosarcomas had positive lymph nodes, in contrast to rhabdomyosarcomas and malignant fibrous histiocytomas with 77.8% and 40% positive lymph nodes, respectively. The 5-year disease-specific survival for women with positive lymph nodes was 0%. CONCLUSIONS: Vulvar sarcomas are heterogeneous with survival highly dependent on the histopathologic subtype. While surgical excision is the mainstay of treatment for all vulvar sarcomas, staging lymphadenectomy should be deferred for (dermato)fibrosarcomas, liposarcomas, and leiomyosarcomas as there were no cases of lymph nodes metastases.

Dedifferentiated transformation in metastatic liposarcoma of orbit and brain.

A liposarcoma of the orbit is extremely rare and a metastatic liposarcoma of the orbit is even rarer. Histologically, metastatic tumors frequently resemble the appearance of the primary lesion or may be less differentiated. In the present paper, we report on a case of a metastatic liposarcoma of the orbit and brain in which dedifferentiated transformation occurred from the primary tumor. The primary liposarcoma was a myxoid liposarcoma in the thigh, which had been resected 6 years ago. The metastatic orbital liposarcoma was poorly differentiated compared to the primary tumor and the tumor in the brain was even more poorly differentiated and anaplastic than the tumor in orbit. The tumor in the orbit and brain was completely removed and postoperative radiotherapy and chemotherapy were performed.

Long-Term Survival According to Histology and Radiologic Response to Preoperative Chemotherapy in 126 Patients Undergoing Resection of Non-GIST Sarcoma Liver Metastases.

BACKGROUND: Non-gastrointestinal stromal tumor sarcomas (NGSs) have heterogeneous histology, and this heterogeneity may lead to uncertainty regarding the prognosis of patients with liver metastases from NGS (NGSLM) and decision regarding their surgical management. Furthermore, the role of preoperative chemotherapy in treatment of NGSLM remains poorly defined. We investigated long-term survival and its correlation to response to preoperative chemotherapy in patients with NGSLM. PATIENTS AND METHOD: Patients who underwent liver resection for NGSLM during 1998-2015 were identified. Clinical, histopathologic, and survival data were analyzed. Multivariate analysis was performed using a Cox proportional hazards model. RESULTS: 126 patients [62 (49%) with leiomyosarcoma] were included. Five-year overall survival (OS) and recurrence-free survival (RFS) rates were 49.3 and 14.9%, respectively. Survival did not differ by histologic subtype, primary tumor location, or use of preoperative or postoperative chemotherapy. NGSLM ≥ 10 cm and extrahepatic metastases at NGSLM diagnosis were the only independent risk factors for OS. In the 83 (66%) patients with metachronous NSGLM, disease-free interval > 6 months was associated with improved OS and RFS. Among the 65 patients (52%) who received preoperative chemotherapy, radiologic response according to Choi criteria specifically was associated with improved OS (p = 0.04), but radiologic response according to RECIST 1.1 criteria was not. CONCLUSIONS: Resection of NGSLM led to a 5-year OS rate of 49%, independent of histologic subtype and primary tumor location. Choi criteria (which take into account tumor density) are superior to RECIST 1.1 in assessing radiologic response and should be used to assess response to preoperative chemotherapy.

Multiple skeletal muscle metastases revealing a cardiac intimal sarcoma.

We report the case of a 59-year-old female with progressive bilateral painful swelling of the thighs. MRI revealed multiple intramuscular necrotic masses with similar morphologic patterns. Whole-body CT and 18-FDG PET-CT scans demonstrated additional hypermetabolic muscular masses and a lobulated lesion within the left atrial cavity. As biopsy of a muscular mass was compatible with a poorly differentiated sarcoma with MDM2 oncogene amplification, two diagnoses were discussed: a dedifferentiated liposarcoma with muscle and heart metastases or a primary cardiac sarcoma, mainly a cardiac intimal sarcoma, with muscular metastases, which was finally confirmed by array-comparative genomic hybridization (aCGH) in a sarcoma reference center. This case emphasizes the potential for intimal sarcoma to disseminate in skeletal muscle prior to any other organ and the need for a genomic approach in addition to classical radiopathologic analyses to distinguish primary from secondary locations facing simultaneous tumors of the heart and skeletal muscles with MDM2 amplification.

Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial.

BACKGROUND: A non-randomised, phase 2 study showed activity and tolerability of eribulin in advanced or metastatic soft-tissue sarcoma. In this phase 3 study, we aimed to compare overall survival in patients with advanced or metastatic soft-tissue sarcoma who received eribulin with that in patients who received dacarbazine (an active control). METHODS: We did this randomised, open-label, phase 3 study across 110 study sites in 22 countries. We enrolled patients aged 18 years or older with intermediate-grade or high-grade advanced liposarcoma or leiomyosarcoma who had received at least two previous systemic regimens for advanced disease (including an anthracycline). Using an interactive voice and web response system, an independent statistician randomly assigned (1:1) patients to receive eribulin mesilate (1·4 mg/m(2) intravenously on days 1 and 8) or dacarbazine (850 mg/m(2), 1000 mg/m(2), or 1200 mg/m(2) [dose dependent on centre and clinician] intravenously on day 1) every 21 days until disease progression. Randomisation was stratified by disease type, geographical region, and number of previous regimens for advanced soft-tissue sarcoma and in blocks of six. Patients and investigators were not masked to treatment assignment. The primary endpoint was overall survival in the intention-to-treat population. The study is registered with ClinicalTrials.gov, number NCT01327885, and is closed to recruitment, but treatment and follow-up continue. FINDINGS: Between March 10, 2011 and May 22, 2013, we randomly assigned patients to eribulin (n=228) or dacarbazine (n=224). Overall survival was significantly improved in patients assigned to eribulin compared with those assigned to dacarbazine (median 13·5 months [95% CI 10·9-15·6] vs 11·5 months [9·6-13·0]; hazard ratio 0·77 [95% CI 0·62-0·95]; p=0·0169). Treatment-emergent adverse events occurred in 224 (99%) of 226 patients who received eribulin and 218 (97%) of 224 who received dacarbazine. Grade 3 or higher adverse events were more common in patients who received eribulin (152 [67%]) than in those who received dacarbazine (126 [56%]), as were deaths (10 [4%] vs 3 [1%]); one death (in the eribulin group) was considered treatment-related by the investigators. INTERPRETATION: Overall survival was improved in patients assigned to eribulin compared with those assigned to an active control, suggesting that eribulin could be a treatment option for advanced soft-tissue sarcoma. FUNDING: Eisai.

[Recurrence of Well Differentiated Intrascrotal Liposarcoma in Retroperitoneum Five Years after Resection : A Case Report].

A 68-year-old man underwent an inguinal orchiectomy for a right testicular tumor and the pathological diagnosis was atypical lipomatous tumor. Nine years later, a resection procedure was performed for local recurrence. Five years after that second surgery, abdominal computed tomography (CT) findings revealed a low density mass 40 mm in size on the back side of the right kidney and enlarged fat in the retroperitoneal space. We performed a laparoscopic tumor resection under a diagnosis of lipoma or liposarcoma recurrence, and the pathological diagnosis was well differentiated liposarcoma. Treatment with pazopanib was started, as a CT examination showed that the tumor remained, after which we performed an open nephroureterectomy and resected the remaining tumor portion. Pazopanib treatment was continued and no obvious signs of recurrence were seen at 8 months after the most recent surgery. Although well differentiated liposarcoma usually recurs in the original tumor region, multicentric recurrence in other parts is possible.

[A Case of a Retroperitoneal Liposarcoma with Long-Term Survival after Four Surgical Resections].

Retroperitoneal liposarcoma is a relatively rare tumor. The only established therapy is surgical resection and the tumor often recurs. This paper deals with a case of a retroperitoneal liposarcoma in which frequent surgical resections for recurrent tumors have provided relatively long-term survival for the patient. The patient was a 70-year-old woman who had undergone surgical resection for a right retroperitoneal tumor. The pathological diagnosis was dedifferentiated liposarcoma. Thereafter she experienced frequent recurrences which required 3 surgical resections. By means of positive margin for the last surgery, chemotherapy with eribulin was administered. There has been no recurrence 13 months after the last surgery.

[Epididymal liposarcoma with metastases to the retroperitoneal space and peritoneal cavity: A case report and literature review].

OBJECTIVE: To report a rare case of epididymal liposarcoma with multiple metastases to the retroperitoneal space and peritoneal cavity for the purpose of improving the diagnosis and treatment of the disease. METHODS: We retrospectively analyzed the clinical data about a case of epididymal liposarcoma with multiple metastases to the retroperitoneal space and peritoneal cavity, reviewed relevant literature at home and abroad, and investigated the presentation, diagnosis and treatment of the disease. RESULTS: A 47-year-old male patient with epididymal liposarcoma underwent radical left orchiectomy with high ligation of the spermmatic cord. Ten years later, recurrence and metastasis were observed in the retroperitoneal space with a mass of 15.6 × 9.4 × 25.5 cm occupying an area from the upper pole of the left kidney to the pelvic cavity and enclosing the left kidney and upper ureter, for which the patient received radical resection of the retroperitoneal tumor. However, multiple retroperitoneal and intraperitoneal metastases occurred again 4 years later, which was treated by another surgical resection. The patient died of lung failure a year later. CONCLUSIONS: Epididymal liposarcoma is a rare entity, which can be diagnosed by careful clinical and radiological examinations and confirmed by pathology. For its treatment, radical inguinal orchiectomy should be performed as early as possible, but the roles of adjuvant radiotherapy and chemotherapy remain controversial. Given the high rate of recurrence and metastasis, long-term follow-up is necessitated for the patient postoperatively.

[A Case of Retroperitoneal Liposarcoma That Invaded and Filled the Jejunal Lumen].

A jejunal tumor was found with computed tomography in a 79-year-old man with a history of gastrectomy reconstructed with Billroth II method for gastric ulcers. The tumor with contrast effect extended into the afferent loop and invaded the retroperitoneum in the dorsal side. The tumor occupied the jejunal lumen in endoscopic examination. Malignant spindle cells were found in the biopsy specimen. An increase of MDM2 and CDK4 signals were observed in fluorescence in situ hybridization( FISH). Given the preoperative diagnosis of jejunal liposarcoma, we performed a resection of the tumor with partial jejunectomy and partial gastrectomy. In the pathological examination of the surgical specimen, the majority of the tumor was dedifferentiated sarcoma; relatively highly differentiated sarcoma cells were observed in the retroperitoneal lesion. Therefore, it was suspected that the retroperitoneal-derived liposarcoma had invaded the jejunal lumen.

Cardiac liposarcoma-a review of outcome after surgical resection.

OBJECTIVE: This review was performed to pool the current surgical strategies for cardiac liposarcoma. METHODS: A literature search was performed and all studies published in full-text or abstract forms were eligible for inclusion without applying any language restrictions. Case reports without surgical intervention, reporting noncardiac liposarcoma, animal cases, or review articles were excluded after initial abstract review. Analyzed postoperative outcomes included intraoperative and in-hospital mortality, longest reported survival, and recrudescence. RESULTS: After a critical evaluation 53 unique surgically treated case reports published between the years 1966 and December 2012 were included in this review. Most of the reported cardiac liposarcoma are myxoid (49.1%), pleomorphic liposarcoma occur with a prevalence of 20.8%, and well-differentiated tumors are observed in 13.2%. One-year survival rate increases the more differentiated the tumor is categorized: 54.5% for pleomorphic, 65.4% for myxoid, and 100% for well-differentiated liposarcoma (p = 0.096). CONCLUSION: Total surgical resection of cardiac liposarcoma is the only curative option, as it tends to local and distant recurrence. Therefore, a frequent follow-up examination should be considered.

PSMA and FDG PET/CT Findings in Patient With Dedifferentiated Liposarcoma and Prostate Cancer Metastases.

ABSTRACT: Dedifferentiated liposarcoma is an extremely rare and highly malignant tumor. We demonstrated a case of a 75-year-old man with significantly PSMA-avid and mildly FDG uptake-dedifferentiated liposarcoma in the retroperitoneal area. The double-tracer (PSMA and FDG) PET scans could further contribute to differential diagnosis and the following treatment strategy for patients who were suspected with prostate cancer metastases and other malignant tumors simultaneously.

Metastatic liposarcoma of the heart. Case series and brief literature review.

Secondary cardiac tumors are 20-40 times more frequent than primary lesions. Primary cardiac lesions are represented by myxomas when related to benign tumors, and by sarcomas in terms of malignant disease. Metastases to the heart from liposarcomas are very rare. We present three cases of secondary liposarcomas involving the left atrium, the right atrium, and the pericardium.

[Rare solid pancreatic tumors]. / Rzadko wystepujace lite nowotwory trzustki.

The most common tumor of the pancreas is cancer, which constitutes 85% of all pancreatic neoplasms. Cystic pancreatic tumors comprise 10% of malignancies. No more than 5% of pancreatic tumors are rare solid tumors as: neuroendocrine tumors, gastrointestinal stromal tumors, solid pseudopapillary tumors, pecomas, lymphomas, granulocytic sarcomas, schwannomas, lipomas, liposarcomas and metastases to pancreas. Nowadays, these tumors are diagnosed more commonly due to the developement and accessibility of the diagnostic imaging techniques. Moreover, the treatment and management of rare solid pancreatic tumors often differs from the management in pancreatic cancer what makes the differential diagnosis difficult and responsible challenge. The main purpose of this article is to present an actual data of epidemiology, clinical presentation, management and treatment of rare solid pancreatic tumors according to recent literature and self experience.

Advanced well-differentiated/dedifferentiated liposarcomas: role of chemotherapy and survival.

BACKGROUND: Data regarding the role of systemic therapy in patients with advanced well-differentiated/dedifferentiated liposarcomas (WDLPS/DDLPS) are limited. METHODS: From 2000 to 2010, 208 patients with advanced WDLPS/DDLPS received chemotherapy in 11 participating institutions. Clinical and pathological data were collected by reviewing medical records. RESULTS: Median age was 63 years (range 32-84). Combination chemotherapy was delivered in 85 cases (41%) and single agent in 123 cases (59%), respectively. One hundred and seventy-one patients (82%) received an anthracycline-containing regimen. Using RECIST, objective response was observed in 21 patients (12%), all treated with anthracyclines. Median progression-free survival (PFS) was 4.6 months [95% confidence interval (CI) 3.3-5.9]. On multivariate analysis, age and performance status (PS) were the sole factors significantly associated with poor PFS. Median overall survival (OS) was 15.2 months (95% CI 11.8 -18.7). On multivariate analysis, grade and PS were the sole factors significantly associated with OS. CONCLUSIONS: Chemotherapy was associated with clinical benefit in 46% of patients with advanced WDLPS/DDLPS. OS remains poor, even though visceral metastatic disease is less frequent than in other sarcomas.

Pleomorphic liposarcoma: A clinicopathologic study of 20 FNA cases.

BACKGROUND: Pleomorphic liposarcoma (PLPS) is the least common but most aggressive of all forms of liposarcoma (LPS). Its diagnosis relies on the recognition of pleomorphic lipoblasts (PLBs), whose numbers vary considerably. Because few large fine-needle aspiration (FNA) biopsy studies exist, the authors review their experience with PLPS. METHODS: The authors' cytopathology files were searched for PLPS with histopathologic verification. FNA biopsy smears were performed via standard techniques. RESULTS: Twenty cases from 20 patients (male/female ratio, 2.3/1; age range, 22-77 years; mean age, 58 years) met the inclusion criteria. All had tissue confirmation. Biopsy sites included the following: thigh (11 [55%]), upper extremity (4 [20%]), axilla (2 [10%]), neck (1 [5%]), chest wall (1 [5%]), and mediastinum (1 [5%]). Aspirates were from primary (17 [85%]), locally recurrent (2 [10%]), and metastatic neoplasms (1 [5%]). The FNA diagnoses were PLPS (10 [50%]), myxofibrosarcoma (4 [20%]), LPS (2 [10%]), sarcoma (2 [10%]), and high-grade malignant neoplasm (2 [10%]). Smears showed thick cell clusters and dissociated single forms. Pleomorphic, epithelioid, and bizarre cell/nuclear shapes were common. PLBs were absent, rare, or unnoticed in 45%. In 25%, smears dominated by myxoid stroma were diagnosed as high-grade myxofibrosarcoma or myxoid LPS. Ancillary testing performed in 5 cases had limited diagnostic efficacy. CONCLUSIONS: FNA biopsy of PLPS, although able to successfully recognize malignancy, suffers from a sampling bias due to an inability to capture or recognize PLBs in a significant proportion of cases secondary to the heterogeneous composition of this neoplasm.

Well-differentiated hand liposarcoma with bone metastases treated successfully with zoledronic Acid.

BACKGROUND: Well-differentiated liposarcomas of the upper extremities, such as the hand or forearm, are extremely rare and are commonly misdiagnosed and considered benign. They are frequently reported as having low metastatic potential. CASE REPORT: We report the case of a 58-year-old man with well-differentiated hand liposarcoma and diffuse bone metastases. The patient initially presented with a tumor of the thenar eminence and was treated with marginal surgical excision and external beam irradiation. 4 months after this treatment, soft tissue local recurrence and skeletal metastases were detected. Zoledronic acid treatment (4 mg per month) was initiated, and both the tumor and the metastases regressed. The patient remains cancer-free 3.5 years after diagnosis of the metastatic disease. CONCLUSION: Significant metastatic disease can develop in patients with well-differentiated liposarcomas. The beneficial effect of zoledronic acid observed in this case may be related to its anticancer properties, as described in phase III trials in various tumor types.

Giant metastatic cardiac and abdominal liposarcomas causing hemodynamic deterioration.

Myxoid liposarcoma usually develops in the retroperitoneum or deep soft tissues of extremities. Synchronous cardiac and abdominal metastases are extremely rare. We describe the case of a 63-year-old man, with a history of a thigh liposarcoma, treated by resection, chemotherapy and postoperative radiotherapy 13 years ago. Patient now presented with an intracardial, a pericardial and an abdominal mass, identified as metastatic myxoid liposarcomas. He eventually underwent successful emergency sternotomy and subsequent laparotomy for tumor resection.