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.

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.

Liver resection for sarcoma metastases: A systematic review and experience from two European centres.

INTRODUCTION: Studies reporting outcomes of liver resection for sarcoma metastases (LRSM) typically include gastrointestinal stromal tumours (GIST), or pooled analyses of "non-colorectal liver metastases", which do not reflect the subgroup of patients with sarcomatous liver metastases. This study aimed to perform a systematic review to evaluate oncological and surgical outcomes in patients undergoing LRSM, and to report new data from two tertiary institutions. METHODS: MEDLINE and the Cochrane Library were searched for studies reporting oncological and surgical outcomes after LRSM, following PRISMA guidelines. Studies reporting liver resection for GIST were excluded. The resulting studies were pooled, with data from two European centres. RESULTS: Six studies of LSRM were included, comprising 212 patients from previously reported series and 24 patients from ours, with median follow-up times of 18-53 months. Postoperative mortality rates ranged from 0 to 9%, and the pooled overall survival (OS) was 89% (95% CI: 83-96%), and 31% (95% CI: 14-47%) at one and five years, respectively (median: 36 months). The presence of synchronous extra-hepatic metastases was found to be a significant risk factor for shorter OS in two cohorts, with hazard ratios of 3.7 (p < 0.001) and 9.1 (p = 0.016), respectively. The largest reported series also found larger metastases (≥100 mm), lack of response to chemotherapy and a shorter disease-free interval to be associated with significantly shorter OS after LSRM. CONCLUSIONS: Patients undergoing LRSM with negative prognostic factors such as the presence of extra-hepatic metastases are unlikely to benefit from surgery. Acceptable medium- and long-term survival may be achievable in highly selected patients.

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.

Primary gastrointestinal liposarcoma-a clinicopathological study of 8 cases of a rare entity.

Primary gastrointestinal liposarcoma is rare, and information regarding this entity is largely based on single case studies. We report on 8 patients with primary liposarcoma of the gastrointestinal tract and review the pertinent literature. The cohort includes 6 men and 2 women who ranged in age from 51 to 81 years (median 68.5). Two tumors arose in the stomach, 4 in the small intestine, and 2 in the large intestine. Tumors ranged in size from 2.5 to 14.5 cm (median 7 cm), originated in the submucosa or muscularis propria of the intestinal wall, and frequently protruded into the bowel lumen, resulting in mucosal ulceration and luminal obstruction. Six tumors were dedifferentiated liposarcomas, and 2 were well-differentiated liposarcoma. Surgical excision was performed on all tumors except for 1 case of dedifferentiated liposarcoma. On follow-up, 1 patient with dedifferentiated liposarcoma developed a lytic sacral lesion suspicious for metastasis 4 months after resection of the primary, and another underwent marginal resection and presented with recurrence 4 years later, had tumor re-resection, and was considered disease-free at 6 weeks postsurgery. A third patient with dedifferentiated liposarcoma was alive with unknown disease status at 17 months following surgery, and another patient with dedifferentiated liposarcoma was alive without evidence of disease at 30 months following surgery. No follow-up information on the remaining patients is available. Overall, liposarcomas of the intestinal tract are most frequently high-grade dedifferentiated tumors that are biologically aggressive and require surgical excision with widely negative margins to help reduce the risk of local recurrence and dissemination. Important in the differential diagnosis is malignant gastrointestinal stromal tumor. Care must be taken not to misdiagnose one entity for the other because the correct diagnosis carries important therapeutic implications.

Cerebral Liposarcoma Embolus From Heart Metastasis Successfully Treated by Endovascular Extraction Followed by Cardiac Surgery.

The current case presents a patient with a tumor emboli stroke as a presenting symptom of a liposarcoma metastasis to the heart that was treated successfully with endovascular mechanical retrieval, followed by subsequent cardiac surgery. The patient is still alive, under chemotherapy treatment, 3 years following the interventions. This scenario should be considered as a part of the differential diagnosis of oncology patients presenting with new central neurological symptoms. This active approach can be an effective treatment if the patient is fit and there is no evidence of widespread disease.

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.

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.

[RETROPERITONEAL LIPOSARCOMA WITH MULTIPLE RECURRENCE OF LUNG METASTASES TREATED BY MULTIMODAL THERAPY CENTERING ON THE OPERATION: A CASE REPORT].

A 34-year-old man presented with scrotal pain and slight fever. The scrotal pain was improved by the treatment of antibiotics, but the slight fever remained and an abdominal protuberance appeared. Computed tomography showed a 22 cm abdominal tumor with lipid density. He was then referred to our hospital. He was diagnosed as retroperitoneal liposarcoma and a surgical resection was performed for retroperitoneal tumor and surrounding organs. Histopathological diagnosis was dedifferentiated liposarcoma. 3 months after surgery, a PET/CT scan showed multiple lung metastases. We treated the patient with AI therapy by doxorubicin and ifosfamide. After 6 courses were performed, a complete response was achieved. 30months after the initial surgery, a PET/CT scan showed there was just one metastasis which was in the left lung. Thoracoscopic lung tumor resection was performed. Histopathological diagnosis was metastatic dedifferentiated liposarcoma. As adjuvant therapy, we treated with IE therapy by ifosfamide and VP-16. 3 courses were performed. 3 years and 6 months after the first surgery, he has had no recurrence up to the present day.

Dedifferentiated Liposarcoma With Epithelioid/Epithelial Features.

Dedifferentiated liposarcoma (DDLPS) demonstrates a variety of growth patterns, and their histologic resemblance to other spindle cell mesenchymal tumors has been widely recognized. However, epithelioid morphology in DDLPS has only rarely been documented. Here, we report 6 cases of DDLPS with striking epithelioid/epithelial features. The patients were 5 men and 1 woman with a median age of 61 years. All tumors were located in the internal trunk. During follow-up of 1 to 41 months, local recurrence, distant metastases, and tumor-related death occurred in 4, 2, and 4 patients, respectively. Beside well-differentiated liposarcoma component and conventional high-grade spindle cell morphology, all tumors focally exhibited growth comprising small or large epithelioid cells in diffuse or sheet-like proliferation. Rhabdoid cells were present in 2 cases. All 5 tumors tested harbored MDM2 amplification. Cytokeratin and/or epithelial membrane antigen were at least focally positive in all 5 tumors tested. One case contained a small focus of novel heterologous epithelial differentiation with acinar structures, wherein cytokeratin, MOC31, and claudin-4 were diffusely expressed and H3K27me3 expression was lost. DDLPS with epithelioid/epithelial features may lead to misdiagnosis of carcinoma or mesothelioma, and their diagnosis should be based on correlation with clinicopathologic and molecular findings. The epithelioid morphology in DDLPS may suggest an aggressive behavior based on this small series. In addition, we document 2 cases of MDM2-amplified undifferentiated neoplasm with epithelioid features in the internal trunk that lacked association with well-differentiated liposarcoma histology and showed rapid clinical course. Whether these latter tumors belong to DDLPS with epithelioid features requires further study.

Metastatic Liposarcoma of the Skull Base: A Case Report and Review of Literature.

BACKGROUND AND IMPORTANCE: Myxoid liposarcoma is not an uncommon form of sarcoma. However, it usually affects the lower extremity long bones. Scapular involvement is extremely rare, as is a metastasis to the parasellar region. We present a case of liposarcoma of the skull base originating in the scapular region and metastasizing to the sellar and parasellar regions and provide a review of the pertinent literature. CLINICAL PRESENTATION: A 43-year-old female patient diagnosed with left scapular myxoid liposarcoma was treated with surgical resection. She had clear resection margins and was treated pre- and postoperatively with radiotherapy to the region. She remained asymptomatic for 2 years following surgery, after which she abruptly developed diplopia with right lateral gaze. There were no symptoms of raised intracranial pressure or impaired vision. Her examination was normal apart from complete right sixth nerve palsy. Imaging studies of the brain showed a large mass in the clivus eroding into the floor of the sella, encircling the right internal carotid artery in the cavernous sinus. The mass also displaced the sellar contents superiorly. An endonasal, endoscopic skull base approach was undertaken, and a subtotal resection was performed in an effort to avoid multiple cranial nerve pareses. CONCLUSION: Our literature search revealed that this case report is the first to document liposarcoma metastasis to the skull base originating from the scapular region. Subtotal surgical resection resulted in minimal improvement of the patient's sixth nerve palsy. Postoperative radiation was undertaken. A multidisciplinary approach on an individual patient basis is recommended.

[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.

[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 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.

[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.