Retroperitoneal liposarcoma presenting a indirect inguinal hernia

A 60-year-old man was admitted to our hospital with a right inguinal swelling that had been growing in size without any pain for 7 months. We diagnosed the growth as a right inguinal hernia and operated on him. The growth, however, was found to be a tumor it situated along the spermatic cord and testicular vessels. We diagnosed it as a lipoma. The tumor was resected near part of the internal inguinal ring. Histopathological diagnosis showed well-differentiated liposarcoma of the sclerosing type. Postoperative computed tomography (CT) revealed a large residual tumor in the retroperitoneum. We believed that the tumor was a retroperitoneal liposarcoma and that it developed in the inguinal region. The residue of the liposarcoma was resected onto the right inguinal tract. A periodic follow up has been performed and no evidence of recurrence or metastasis has been seen in the 4 years and 9 months since the second surgery. No adjuvant therapy was performed. Inguinal liposarcomas are relatively rare and in most cases these tumors are thought to originate in the spermatic cord. The origin of the tumor is believed to be the retroperitoneum.     

Retroperitoneal extraskeletal myxoid chondrosarcoma

INTRODUCTION: Extraskeletal myxoid chondrosarcoma is a rare soft tissue tumor included by WHO in the group of tumors of uncertain differentiation because well formed cartilage is rarely seen in its setting; moreover some of the immunophenotype features of the tumor are still controversial. The reciprocal translocation t(9;22)(q22;q12) has recently been identified in many cases, and this chromosomal aberration is currently considered high specific of this tumor. CASE REPORT: We report the case of a 75-year-old female submitted to radical nephrectomy for a voluminous kidney tumor with radiological evidence of extrarenal extension. RESULTS: Grossly the lesion was set in the perirenal fat tissue. Histological examination revealed a malignant mesenchimal tumor with myxoid f eatures and areas of chondroid differentiation consistent with the diagnosis of extraskeletal myxoid chondrosarcoma. According with the diagnosis the tumor showed focal positivity to NSE by immunohistochemistry. DISCUSSION: Differentiated cartilage is seen in extraskeletal myxoid chondrosarcoma in about 30% of the cases. Our case is then interesting not only for the rare site of tumor location but also for the extension of chondroid differentiation areas found on histologic examination in the tumor setting. Moreover immunohistochemistry showed focal positivity of the neoplastic cells for NSE, according with recent studies demonstrating expression of some neural/neuroendocrine markers in extrascheletal myxoid chondrosarcoma.     

Retroperitoneal well-differentiated inflammatory liposarcoma

Inflammatory liposarcoma represents a rare variant of well-differentiated liposarcoma in which a chronic inflammatory infiltrate predominates simulating the appearance of malignant lymphoma and other nonneoplastic lesions. A case of this lesion occurring in the retroperitoneum was described in 43-year-old man. The differential diagnosis, especially from lymphoma and non neoplastic lesions such as inflammatory myofibroblastic tumor and Castelman disease are discussed.     

Dedifferentiated myxoid liposarcoma: a clinicopathological study suggesting a closer relationship between myxoid and well-differentiated liposarcoma

Myxoid/round cell liposarcoma is arguably the commonest type of liposarcoma occurring in the extremities and may show gradual progression from low-grade, pure myxoid liposarcoma to high-grade round cell liposarcoma. Rarely myxoid/round cell liposarcoma is associated with areas of well-differentiated or pleomorphic liposarcoma (mixed liposarcoma). We describe the clinicopathological features of three unusual myxoid/round cell liposarcomas which showed morphological features of de novo dedifferentiation. All patients were male and were aged 66, 70 and 76 years, respectively. One lesion each arose in the retroperitoneum, inguinal region and peritoneal cavity. Histologically, in one case the myxoid/round cell component was juxtaposed to a high-grade non-lipogenic component resembling non-pleomorphic storiform 'malignant fibrous histiocytoma' ('MFH'), one case showed a combination of myxoid liposarcoma and a high-grade myxofibrosarcoma-like component (so-called myxoid 'MFH'), and in the third case, a well-differentiated myxoid liposarcoma with a discontinuous micronodular pattern of dedifferentiation was seen. Follow-up information of 30, 28 and 26 months revealed two recurrences each in two patients. These patients died of postoperative pulmonary embolism and abdominal haemorrhage, respectively; systemic metastases were not noted. These cases demonstrate that myxoid/round cell liposarcoma can show, albeit very rarely, histological features of dedifferentiation. Cases like these, combined with the occurrence of mixed-type liposarcoma (well-differentiated/myxoid liposarcoma) and the vicinity of chromosomal regions involved by specific karyotypic aberrations in these tumours, suggest that myxoid/round cell liposarcoma and well-differentiated liposarcoma (including its dedifferentiated variant) are more closely related in biological terms than is generally believed.     

心包原发性黏液型脂肪肉瘤一例

患者男,24岁,右脑隐痛4年,加剧4d,于1997年7月14日入院。患者自1994年起右胸隐痛,活动后尤甚,入院前4d疼痛加剧,不得不下蹲以图缓解,疼痛呈持续性向右肋及右肩背处放射,     

Cardiac encasement by metastatic myxoid liposarcoma

Isolated cardiac metastasis from a primary liposarcoma of noncardiac origin is a rare occurrence. A patient who presented with biventricular failure and constrictive hemodynamics years after successful resection of a primary liposarcoma of the thigh is described. Extensive cardiac encasement by tumor was suspected on diagnostic imaging. Hemodynamic instability and multiorgan failure necessitated urgent exploratory sternotomy. The patient died intraoperatively. Extensive metastatic sarcoma limited to the heart was confirmed during surgical procedure. This case suggests that in clinical and pathological investigation of a cardiac mass, knowledge of previous extracardiac involvement with soft tissue sarcoma is essential.     

Paratesticular myxoid liposarcoma: a case report

Liposarcoma of the paratesticular region is a rare malignant soft tissue neoplasm. Out of all the possible sites of liposarcoma, the incidence in spermatic cord is rare, accounting for only 5% of these neoplasms. Among the various subtypes of liposarcoma described in the paratesticular region, myxoid variant of liposarcoma is seen very rarely. One such case is we ported here for its rarity and also to draw attention to the fact that, early diagnosis and complete excision carry better prognostic value.     

Retroperitoneal liposarcoma in an adult patient with Down syndrome

Retroperitoneal liposarcoma is a rare solid tumour of mesenchymal origin with an incidence of 2.5 per million population. We report what is, to the best of our knowledge, the first case in the English literature of retroperitoneal liposarcoma in an adult patient with Down syndrome. The tumour was surgically resected with no use of adjuvant chemotherapy or radiation. No recurrence was found at follow-up 2 months postoperatively. Clinicians should consider retroperitoneal liposarcoma in the differential diagnosis of abdominal distention in adult patients with Down syndrome.     

A case of myxoid liposarcoma of the breast

A 70-year-old woman visited a local hospital complaining of a nodulein the right breast, present since 1 month. She was referred to our hospital for further evaluation. Following mammotome (MMT) biopsy, the nodule was diagnosed as myxoid/round cell liposarcoma. She underwent total mastectomy of the right breast. Histological analysis indicated that the tumor was almost entirely composed of proliferating small round mesenchymal cells in amyxoid matrix background with capillary-like vessels with partial necrosis (<10%). Immunohistochemically, p53 positive cells were seen focally (<1%) only, and the Ki-67 labeling index was approximately 20%. Sincethe surgical margin was histologically positive despite pathologic findings of high-grade malignancy, adjuvant treatment involving local radiation therapy (60Gy) was administered. The patient was free from any symptoms of local recurrence and metastases 1 year and 8 months after surgery.     

Lipoblastoma presenting as a rapidly growing paravertebral mass and masquerading as myxoid liposarcoma on fine needle aspiration cytology

Lipoblastoma is a peculiar variant of lipoma occurring almost exclusively during infancy and early childhood. It is found most commonly in the upper and lower extremities; less common sites are head and neck, trunk, mediastinum, and retroperitoneum. It has a greater predilection for boys and commonly presents as a slowly growing soft‐tissue mass. We present here the case of a five‐ year old female child with a lipoblastoma presenting as a paravertebral mass in the right lower back which progressed rapidly in the previous six months causing diagnostic difficulty on fine needle aspiration cytology. Diagn. Cytopathol. 2016;44:426–429. © 2016 Wiley Periodicals, Inc.     

Retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components: a case report

We report a rare case of recurrent retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components. An 82-year-old male diagnosed with recurrent retroperitoneal liposarcoma underwent a tumor resection. Histologically, osseous matrix with osteoid and mature hyaline cartilaginous tissues with high cellularity were observed in a fibrous background through most of the tumor, and scattered MDM2- and CDK4-positive atypical hyperchromatic stromal cells were detected surrounding the dedifferentiated areas. Dedifferentiation occurs in up to 10% of well-differentiated liposarcomas, frequently resembling a malignant fibrous histiocytoma-like pleomorphic sarcoma. In contrast, divergent differentiation with osteosarcomatous components is considered to be extremely rare.     

Myxoid and round cell liposarcoma: a spectrum of myxoid adipocytic neoplasia.

Myxoid and round cell liposarcoma accounts for about 30% to 35% of all liposarcomas and, even if still classified by the World Health Organization (WHO) as 2 distinct subtypes, share both clinical and morphologic features. Lesions combining both patterns are frequent and wide agreement exists in considering round cell liposarcoma as the high grade counterpart of myxoid liposarcoma. Furthermore, myxoid and round cell liposarcoma share the same characteristic chromosome change represented most frequently by a reciprocal translocation t(12;16)(q13;p11) that fuses the CHOP gene with the TLS gene. Clinically, myxoid and round cell liposarcoma tend to occur in the limbs with a peak incidence ranging between the third and the fifth decade and exhibit overall a metastatic rate of approximately 30%. A peculiar tendency to metastasize to the soft tissue is observed that should not be interpreted as multicentricity. Microscopically, purely myxoid liposarcoma is composed by a hypocellular spindle cell proliferation set in a myxoid background and associated with a varying number of monovacuolated lipoblasts. The most helpful morphologic clue is represented by the presence of a thin-walled capillary network organized in a plexiform pattern. The most important morphologic variation observed in myxoid liposarcoma is represented by the occurrence of hypercellular areas that may exhibits an undifferentiated round cell morphology. On the basis of the percentage of hypercellularity/round cell formation, a myxoid/round cell liposarcoma (more than 25% hypercellular/round cell areas) and a round cell liposarcoma (more than 75% hypercellular/round cell areas) are somewhat arbitrarily recognized. Both the recognition and the quantification of hypercellular/round cell areas represents a crucial step in the evaluation of this liposarcoma subtype because hypercellularity appears to correlate with the clinical outcome. In consideration of the intrinsic difficulty in establishing accurately the percentage of high grade areas as well as of application of different cut off values, it appears safer to consider any amount of hypercellularity as prognostically relevant. Careful as well as extensive sampling is mandatory to permit detection of the smallest amount of hypercellularity. The differential diagnosis of myxoid liposarcoma includes benign lesions, such as myxoid spindle cell lipoma, intramuscular myxoma and lipoblastoma, and malignant ones such as low grade myxofibrosarcoma, and extraskeletal myxoid chondrosarcoma. In consideration of the great morphologic variability, the application of both immunohistochemistry and genetics has proved helpful in sorting out the more challenging cases.     

Fine-needle aspiration smears from lipomatous hemangiopericytoma need not be confused with myxoid liposarcoma

Lipomatous hemangiopericytoma (LHPC) is a rare benign soft tissue tumor that may mimic soft tissue sarcoma clinically. Despite the fact that LHPC shares most of the histologic features with solitary fibrous tumor, it may be occasionally misdiagnosed as myxoid liposarcoma or some other type of spindle-cell sarcoma. While some studies have addressed the histological features and origin of LHPC, no reports of cytological findings have been published. The author describes the cytological features of LHPC in two patients with correlations to histological and clinical findings. One of aspirates was misdiagnosed as being myxoid liposarcoma, while the other one was correctly diagnosed by a combination of FNAB and core needle biopsy (CNB). Cytological evaluation of LHPC can be problematic due to its rarity and to its similarity with other spindle-cell or fatty tumors. Correct diagnosis requires an awareness of cytologic features of this rare tumor and the use of ancillary studies.     

Trisomy 8 as a nonrandom secondary change in myxoid liposarcoma

We report the cytogenetic findings in 5 cases of myxoid liposarcoma following short-term culture. In all 5 tumors a t(12:16)(q13:p11), characteristic of the myxoid form of liposarcoma, was observed. Trisomy 8 was present in two tumors as the only additional change and in a third in addition to other abnormalities. In the other two tumors the t(12:16) was present as the sole change. Three other myxoid liposarcomas with trisomy 8 as an additional aberration have been reported, suggesting that it could represent a non-random secondary event in these tumors.     

An unusual presentation of myxoid liposarcoma: a case report

A 50 year old female had a myxoid liposarcoma of left thigh that was widely excised. After three years, she presented with a soft tissue mass in right hypochondriac region, which was diagnosed as myxoid liposarcoma on fine needle aspiration cytology (FNAC) and was confirmed histologically.