Cases reported "Retroperitoneal Neoplasms"

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1/46. Giant retroperitoneal hemangiopericytoma with paraneoplastic gynecomastia. Case report and literature review.

    We report on a 38-year-old patient with a giant retroperitoneal hemangiopericytoma associated with bilateral paraneoplastic gynecomastia, which led to an almost uncontrollable hemorrhage on initial surgery due to the extreme hypervascularity of the process. After angiographic evaluation and superselective embolization, a complete surgical excision of a hemangiopericytoma weighing almost 1,000 g could be achieved. To reduce the risk of recurrence adjuvant radiotherapy with 49 Gy followed. Follow-up of 24 months showed no sign of recurrence on CT scans. To our knowledge this is the first reported case of hemangiopericytoma associated with a paraneoplastic syndrome. The literature and the therapeutic concepts are presented and discussed.
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2/46. Giant pelvic retroperitoneal liposarcoma.

    BACKGROUND: Pelvic retroperitoneal liposarcomas are rare in young women (under 30 years old). We present a case of a giant well-differentiated liposarcoma of the retroperitoneum in a young woman. CASE: A 27-year-old nulligravida presented with rapid abdominal enlargement. Pelvic examination found a huge mass extending from the left adnexa to the epigastric region. At surgery, the pelvic organs were displaced to the right side by a retroperitoneal mass that marginally involved the left fallopian tube. The left ovary, uterus, and right adnexa were not involved and were not removed. No adjuvant irradiation was given. The patient was alive and well 2 years after the operation and was 23 weeks pregnant. CONCLUSION: Gynecologists should be familiar with pelvic retroperitoneal liposarcomas and their treatment.
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3/46. Fine needle aspiration cytology of well-differentiated liposarcoma. A report of two cases.

    BACKGROUND: Well-differentiated liposarcoma is difficult to diagnose on fine needle aspiration cytology (FNAC) smears and may create considerable diagnostic problems. CASES: Males aged 60 and 45 years presented with a swelling in the groin and retroperitoneal region, respectively. FNAC showed large cells with multilobulated nuclei and mature-looking fat tissue. A soft tissue tumor with bizarre cells was diagnosed cytologically in case 1 and liposarcoma in case 2. Histologically, both cases were diagnosed as well-differentiated sclerosing liposarcoma. CONCLUSION: The cytologic diagnosis of well-differentiated liposarcoma should be done with caution, and the sites should be taken into consideration. Deep-seated tumors with large, bizarre, giant cells should have wide excision as they recur more frequently.
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4/46. Retroperitoneal schwannoma presenting as an adrenal tumor.

    A retroperitoneal tumor in the region of the adrenal gland was diagnosed in a 56-year-old woman. The patient had been suffering from a dull abdominal pain for nearly four weeks before consulting her family physician. Ultrasound, CT and MRI scans revealed a giant tumor of the right adrenal gland. Endocrine activity could not be demonstrated. The size of the tumor was suggestive of an adrenal carcinoma. The patient was referred for adrenalectomy and complete exstirpation of the retroperitoneal mass. The histological examination revealed characteristical findings of a benign schwannoma.
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5/46. A giant ectopic ovary.

    A young woman with a congenital chest-wall defect presented with abdominal pain, irregular menses, and a palpable mass at the defect. The mass, removed laparoscopically, was found to be a functioning ovary that tracked along the retroperitoneum, reflecting its embryologic origin.
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6/46. Fine needle aspiration cytology of a dedifferentiated liposarcoma: report of a case with histologic and immunohistochemical follow-up.

    BACKGROUND: Dedifferentiation is a histologic progression of a neoplasm from low grade to high grade histology. It occurs in tumors of the retroperitoneum and in those undergoing treatment. This usually occurs in the setting of radiation or chemotherapy or as a spontaneous process over a long period. The features of dedifferentiation can be toward any mesenchymal element of the underlying neoplastic process. CASE: We report the cytologic features of a dedifferentiated liposarcoma arising in a 76-year-old man who had a history of well-differentiated liposarcoma. Papanicolaou- and Diff-Quik-stained smears from a radiologically guided fine needle aspiration biopsy showed a hypercellular sample. The smears showed a mixed population of cells. There were multinucleated, pleomorphic giant cells with abundant cytoplasm, smaller clusters of cells with a high nuclear/cytoplasmic ratio and cells with spindled and elongated nuclear features. The follow-up surgical resection specimen showed a dedifferentiated liposarcoma with strong and diffuse immunoreactivity to vimentin, desmin and CD68 in the large, pleomorphic cells; focal and weak immunoreactivity to smooth muscle actin and S-100 in these cells; and strong and focal immunoreactivity to desmin, smooth muscle actin and muscle-specific actin in the spindle cells. This supports the dedifferentiated components of this tumor to be of fibrohistiocytic and leiomyosarcomatous differentiation. CONCLUSION: Dedifferentiation of a well-differentiated liposarcoma should be entertained in the setting of a mass lesion in the retroperitoneum in patients with prior histories of well-differentiated liposarcoma. The radiologic features of a particular neoplastic process can be very helpful in determining the nature of this process.
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7/46. Two cases of melanose neurocutanee with development of malignant melanoma: a microspectrophotometric and electron microscopic study.

    Two cases of neurocutaneous melanosis with development of malignant melanoma in the Japanese are presented. The first case was a 4-year-old boy in whom a retroperitoneal melanoma appeared with giant nevi, and cerebral and spinal melanosis. The second case was a 39-year-old man, in whom a primary leptomeningeal melanoma developed with leptomeningeal melanosis and smaller pigmented nevi. Microspectrophotometric and electron microscopic studies were made on the neoplastic and non-neoplastic melanotic tissues to elucidate the histogenesis of this rare disorder. Two different patterns of nuclear dna histograms, corresponding to melanosis and melanoma, were obtained by microspectrophotometry, Considerable variation in the ultrastructure of the melanocytes was seen by electron microscopy.
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8/46. Giant cystic pheochromocytoma located in the renal hilus.

    A malignant tumor in the past medical history of a patient often makes the differential diagnosis of a second tumor more difficult, especially if one of the tumors does not show its characteristic features. The authors report a case of a 55-year-old male who presented with a malignant melanoma on his left shoulder. A retroperitoneal giant cystic mass, 200 mm in diameter, was found incidentally. Adrenal origin was ruled out by imaging techniques. The absence of typical clinical symptoms made a correct preoperative diagnosis unlikely, and severe cardiovascular complications set in during surgery. Considering the characteristics of the cutaneous malignant melanoma, the metastatic origin of the giant retroperitoneal tumor was not likely either. During surgery the left kidney, with a cystic tumor located in the hilus, was removed. The postoperative pathologic diagnosis was pheochromocytoma located in the hilus of the left kidney.
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9/46. A case of giant retroperitoneal liposarcoma.

    A 44-year-old female patient presented to us with fever and increased abdominal girth. A CT scan showed a very large retroperitoneal mass displacing the right kidney. At operation three enormous retroperitoneal tumours displacing the ascending colon and the right kidney and compressing the inferior vena cava were removed. Histopathological examination revealed a myxoid retroperitoneal liposarcoma.
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10/46. Retroperitoneal giant schwannomas: report on two cases and review of the literature.

    The occurrence of massive retroperitoneal schwannomas is extremely rare and their presence may only be expressed by insidious onset of non-specific and misleading symptoms with a predominance of lower back pain. MRI scan as the imaging procedure of choice will demonstrate the tumour location and its relation to the surrounding structures, but due to heterogeneity and degeneration in some tumours, it may mimic malignancy. Hence tissue sampling through needle biopsies are essential to verify the diagnosis prior to surgery. Tumour excision in toto is considered the treatment of choice, but it can be hazardous especially if the tumour is adherent to the presacral venous plexus. Severe bleeding complications due to the damage of venous structures have to be encountered, and establishing lasting haemostasis may pose considerable difficulties. Hence surgery should be attempted with full precautions, and preoperative counseling of the patient. If malignancy can safely be excluded, laparoscopic piecemeal excision should be considered as an alternative treatment as recurrence is unlikely. Definition of the originating nerve might not always be possible and a minor degree of neurological impairment has therefore to be anticipated.
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