Differentiated liposarcoma with a paraganglioma-like histologic pattern: A case report and review of the literature
Department of Pathology, University of Manitoba, Winnipeg.Archives of pathology & laboratory medicine (Impact Factor: 2.84). 08/2004; 128(7):788-91. DOI: 10.1043/1543-2165(2004)128<788:DLWAPH>2.0.CO;2
A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 x 32 x 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.
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ABSTRACT: Sixty-one cases of neoplasms composed wholly or in part of atypical lipomatous tumor were reviewed. Minimum follow-up was 10 years. The cases were divided into 4 groups based on the findings in the initial excision specimen: conventional atypical lipomatous tumor (n=15), cellular atypical lipomatous tumor (n=21), dedifferentiated liposarcoma (n=24), and atypical lipomatous tumor with a pleomorphic liposarcoma-like component (n=1). The term "cellular atypical lipomatous tumor" was applied to atypical lipomatous tumors having areas of increased cellularity that when non-lipogenic lacked the 5 mitotic figures per 10 high-power fields (maximal rate) required for a dedifferentiated component and when lipogenic fell short of being truly pleomorphic liposarcoma-like. Myxoid regions within this spectrum sometimes had prominent or even plexiform vascularity, creating a resemblance to myxoid liposarcoma especially when interspersed small fat cells were present. The most important prognostic factor was tumor location, as none of the 12 patients with a subcutaneous or intramuscular neoplasm died of tumor. Among the 49 patients with neoplasms of central body sites (mostly retroperitoneum), those with dedifferentiated liposarcoma had significantly shorter survival (median 77 mo) than those with cellular (median 142 mo) or conventional (median 209 mo) atypical lipomatous tumor, whereas there was no statistically significant difference between the latter 2 categories. Patients with atypical lipomatous tumor (either cellular or conventional) in central body sites had significantly shorter survival if the tumor transformed into dedifferentiated liposarcoma in recurrence, and, conversely, those with central body site dedifferentiated liposarcoma had significantly longer survival if it recurred as atypical lipomatous tumor. Metastasis (7 cases) occurred only when the initial specimen or a recurrence demonstrated dedifferentiated liposarcoma.
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ABSTRACT: Dedifferentiated liposarcoma with peculiar meningothelial-like whorling pattern and metaplastic bone formation (DDLMB) is an unusual morphologic entity that is characterized by an atypical lipomatous tumor/well-differentiated liposarcoma with epithelioid or spindle cells concentrically arranged into meningothelial-like "whorls," and mature bone trabeculae rimmed by reactive osteoblasts. We recently experienced 2 cases of DDLMB, one in a 64-year-old male patient with a painless right groin mass and another in a 42-year-old female patient with a painless right abdominal mass. The size of the tumors was 3.5 and 18 cm; and the tumors were located in the right scrotal sac and retroperitoneum in case 1 and case 2, respectively. Under the initial clinical diagnosis of cord lipoma in case 1 and high-grade sarcoma in case 2, the masses were removed. The cut surfaces of the masses were well circumscribed with encapsulation, red-tan, firm, and multinodular. Microscopically, the tumors consisted of atypical lipomatous tumor/well-differentiated liposarcoma with meningothelial-like whorls and metaplastic bone formation in both cases. In addition, the first case showed focal areas of paraganglioma-like pattern; and the second case showed pleomorphic high-grade sarcoma with low-grade myxofibrosarcoma-like areas. Immunohistochemically, the tumor components with meningothelial-like pattern and paraganglioma-like pattern in DDLMB were positive for vimentin and CD56 and negative for pancytokeratin, epithelial membrane antigen (EMA), desmin, and smooth muscle actin. Characteristically, the paraganglioma-like area was immunoreactive for S-100 protein, with a "dot-like" staining pattern. The patient additionally underwent radical orchiectomy in case 1. Review of the literature revealed that only 34 cases of DDLMB have been reported. One case of dedifferentiated liposarcoma with a predominant paraganglioma-like pattern has also been reported in the literature. To our knowledge, case 1 represents the first report of DDLMB with paraganglioma-like pattern. A brief literature review was made with focus on the morphologic variations of DDLMB.
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ABSTRACT: A rare autopsy case of the extremely large retroperitoneal solitary fibrous tumor is reported. A 52-year-old female with a huge abdominal distention was found dead at home. She showed remarkable emaciation. The autopsy revealed a huge retroperitoneal tumor weighing 11.9kg (36×30×20cm in size), which occupied the entire intraperitoneal cavity. Histologically, the tumor consisted of spindle parenchymal cells with fibrous tissues. Immunohistochemically, CD34 was positively stained, whereas S-100, smooth muscle actin, and factor VIII were negative. Her cause of death was diagnosed as emaciation due to the compression of the entire intestine by the tumor. This is a rare case of the extremely large retroperitoneal solitary fibrous tumor, which caused the occasional intestinal obstruction. This disease should be considered in the differential diagnosis of retroperitoneal large tumors that cause accidental deaths in forensic autopsies.
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