Cases reported "Pelvic Neoplasms"

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1/14. Pelvic malignant mixed mesodermal tumor of uncertain origin: a case report.

    Extra-uterine, and especially extragenital, malignant mixed mesodermal tumors (MMMT) are very rare. A large intrapelvic tumor resected from a 56-year-old woman was investigated with morphological and immunohistochemical methods. A large, soft and fragile tumor was located in the pelvic space. The tumor showed high cellularity and was biphasic; it consisted of an admixture of adenocarcinoma and various kinds of sarcomas. The latter were comprised of high-grade endometrial stromal sarcoma, pleomorphic sarcoma, and chondrosarcoma. The pleomorphic sarcoma showed a storiform pattern. The periodic acid-Schiff-positive eosinophilic hyaline droplets and globules in multinucleated giant cells revealed a typical ring-like or peripheral staining for alpha-1-antitrypsin and alpha-1 antichymotrypsin. We considered this case to be pelvic MMMT of uncertain origin, heterologous type.
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2/14. 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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3/14. Treatment of giant ancient pelvic schwannoma.

    A 32-year-old patient presented with urinary retention and chronic constipation. Computerized tomography and magnetic resonance imaging showed a 10 x 11cm encapsulated tumor with cystic areas lying ventral to the sacrum. There was no evidence of invasion of bladder or rectum. At laparotomy, a 10 x 11 cm mass was found in the left pelvis. Final pathology revealed an ancient schwannoma. In most large series, 80% to 90% of the primary retroperitoneal tumors are malignant. Retroperitoneal schwannomas can be benign or malignant, roughly half of the reported cases showed malignancy. Benign schwannomas may arise along the course of any myelinated nerve, with the acoustic neuroma being the most frequent site. Immunostaining showed a strong expression of S-100 protein. The staining for this protein is helpful for differentiation of a benign schwannoma from a malignant peripheral nerve sheath tumor and from other benign spindle cell tumors. The treatment of choice for benign schwannomas is complete excision. recurrence or persistence seems to be associated with incomplete resection, which occurred in 10% of the reported cases. After surgery, the patient had normal erection, normal micturition, and normal defecation but no symptoms of motor and sensory disturbances.
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4/14. Presacral solitary giant neurofibroma without neurofibromatosis type 1 presenting as pelvic mass--case report.

    A 35-year-old woman presented with a solitary neurofibroma in an unusual presacral location without neurofibromatosis manifesting as bilateral chronic sciatica for 2 years. She was initially considered as having a giant right ovarian mass, but was referred with a prediagnosis of solitary giant sacral nerve sheath tumor. The initial differential diagnosis was based on neuroimaging. A right-sided J incision with the extraperitoneal approach provided good exposure and handling of the tumor bed. Almost total excision without neurological deficit was possible. The histological diagnosis was neurofibroma. Benign retroperitoneal neural sheath tumors in patients without von Recklinghausen's disease are quite rare. Intrapelvic tumors are often diagnosed at a later stage. neuroimaging is very helpful to delineate this unusual site and the extent of tumor development, and to determine the appropriate surgical intervention. A clear understanding of retroperitoneal anatomy is essential for safe removal of such tumors. Complete resection is preferred to prevent local recurrence and malignant transformation. Although root section is inevitable, neurological deficit is unlikely.
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5/14. Neurofibromatosis involving the urinary bladder.

    We present two interesting cases of a 24-year-old man and a 14-year-old boy, uncle and nephew, with lower urinary tract symptoms, cafe au lait patches and subcutaneous nodules. ultrasonography and computed tomography scans showed a large, irregular lobulated soft tissue mass between the bladder and sacrum. cystoscopy, laparotomy and biopsies revealed neurofibromatosis involving the urinary bladder. No enlargement of the tumor or upper urinary tract obstruction has occurred during the long-term follow up. We recommend meticulous follow up of patients with giant intrapelvic neurofibromatosis.
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6/14. Giant pelvic retroperitoneal leiomyoma arising from the rectal wall.

    BACKGROUND: Pelvic retroperitoneal leiomyomas arising from the rectal wall are rare. We present a case of a giant retroperitoneal leiomyoma mimicking bilateral solid adnexal masses in a postmenopausal woman. CASE: A 54-year-old postmenopausal woman presented with a large abdominopelvic mass. At surgery, the uterus was displaced anteriorly by a large retroperitoneal mass. The rectosigmoid colon was noted to course through the retroperitoneal mass. The patient underwent complete excision of the retroperitoneal mass along with a rectosigmoid resection of the involved colon with primary reanastomosis. Histopathology showed a leiomyoma arising from the muscularis propria of the rectum wall. CONCLUSION: Retroperitoneal masses that extend into the pelvis may mimic adnexal masses and, therefore, represent a rare finding at gynecologic surgery.
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7/14. Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature.

    AIMS: angiomyolipoma is the most common mesenchymal tumour of the kidney. It has been reported in several other sites outside the kidney, mainly in the liver. We report the first case of atypical pleomorphic angiomyolipoma in a man, arising from the pouch of Douglas and extending to the entire abdominal cavity. methods: A 17-year-old man underwent a complete resection of a giant abdominopelvic mass. The tissue was formalin fixed and paraffin embedded and 4 micro m thick histological sections were stained with haematoxylin-eosin. Immunohistochemical stains for HMB-45, smooth muscle actin, vimentin, calponin, S100 and desmin were performed. Sections for electron microscopy were also prepared. RESULTS: Microscopic examination revealed a neoplasm composed of pleomorphic epithelioid cells with atypical features, immunoreactive for HMB-45, MART-1, actin, vimentin and calponin, while S100 protein and desmin stains were negative. Ultrastructurally, the tumour cells showed prominent nucleoli, vacuolated cytoplasm, and some premelanosomes. A diagnosis of atypical pleomorphic epithelioid angiomyolipoma was then made. CONCLUSIONS: To date five patients with abdominal epithelioid angiomyolipoma have been described in the literature. All were women. Three of the five patients reported developed metastasis, while our patient is still free of disease at 16 months of follow-up. Clear prognostic pathological features have not been identified.
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8/14. Aspiration cytology of malignant fibrous histiocytoma after radiation therapy for carcinoma of the uterine cervix: a case report.

    BACKGROUND: Cytologic reports on malignant fibrous histiocytoma (MFH) following radiation therapy for carcinoma of the uterine cervix are very rare. CASE: A 59-year-old woman presented with slowly increasing pain in the left hip joint. Eight years earlier, she had received radiotherapy at a dosage of 5,000 cGy to the whole pelvis for carcinoma of the uterine cervix. An osteolytic lesion of the pelvic bone was revealed on computed tomography, and a hard tumor was palpable in the left pelvic cavity. Fine needle aspiration (FNA) of the tumor via the left vaginal wall obtained 0.5 mL of yellow fluid consisting of markedly anaplastic and pleomorphic giant cells. Frequent multinucleation and mitoses were observed, although no atypical spindle cells were observed. Immunocytochemistry disclosed vimentin reactivity. An open biopsy of the tumor revealed the histologic and immunohistochemical features of MFH arising in the pelvic cavity. CONCLUSION: FNA of the pelvic lesion via the vaginal wall revealed an MFH in the radiation therapy field. This is one of the few reports dealing with FNA cytology of a postradiation sarcoma in the pelvic cavity.
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9/14. Pelvic Castleman disease mimicking an adnexal tumor.

    BACKGROUND: Castleman disease, or giant lymph node hyperplasia, is a rare cause of lymph node enlargement. Only 11 cases with pelvic localization in women have been reported. CASE: A pelvic mass mimicking an adnexal tumor was detected on a routine examination in a 26-year-old woman. Surprisingly, the pelvic organs were found to be normal at laparotomy. Eight years later, the subject was examined for dyspareunia and secondary infertility that lasted 2 years. A pelvic mass, evidently the one encountered 8 years previously, was detected; it had enlarged only slightly in the intervening time. Surgical exploration of the retropubic space revealed a tumor-like mass attached to the periosteum of the left superior pubic arch. The mass was removed by simple resection. Histologic investigations confirmed a diagnosis of Castleman disease. Three months after the operation, the woman conceived without any further treatment. CONCLUSION: The possibility of Castleman disease should be considered when assessing a pelvic mass. The condition in the retropubic space in women has not been reported previously.
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10/14. Malignant fibrous histiocytoma in chordoma--immunohistochemical evidence of transformation from chordoma to malignant fibrous histiocytoma.

    Sarcomatous transformation in chordoma (dedifferentiated chordoma) is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma (chordoma associated with malignant fibrous histiocytoma) arising from the sacrococcygeal region of a 55-year-old woman. The results of immunohistochemical stain in the chordoma area were strong positive for cytokeratin, epithelial membrane antigen and S-100 protein. The spindle and giant cells in the transitional areas of chordoma and malignant fibrous histiocytoma were positive for cytokeratin and epithelial membrane antigen in addition to vimentin and alpha-1-antichymotrypsin. The spindle and giant cells in the central area of malignant fibrous histiocytoma were negative for cytokeratin and epithelial membrane antigen, but positive for vimentin and alpha-1-antichymotrypsin. This supports the pathogenesis of sarcomatous transformation from chordoma.
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