Cases reported "Neoplasms, Muscle Tissue"

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1/77. Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors.

    Inflammatory myofibroblastic tumor (IMT) is a relatively rare soft tissue tumor. The reactive versus neoplastic pathogenesis of this tumor is unresolved. We found clonal chromosome aberrations involving 2p23 upon metaphase analysis of two IMTs. fluorescence in situ hybridization with a probe flanking the ALK gene at 2p23 demonstrated rearrangement of the probe in both of these cases and in a third case, and immunohistochemistry revealed ALK expression in all three cases. 2p22-24 involvement has been reported previously in four additional cases of IMT. We suggest that chromosomal rearrangements involving 2p23 near or within ALK are recurrent alterations in IMT and that ALK may have a novel role outside its previously recognized realm of lymphoid neoplasms.
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2/77. Pediatric inflammatory bladder tumors: myofibroblastic and eosinophilic subtypes.

    PURPOSE: Benign bladder tumors are rare in children. A number of descriptive terms have previously been used to describe inflammatory tumors with myofibroblastic proliferation or eosinophilic infiltration. We present our experience with these tumors and review the literature. MATERIALS AND methods: We retrospectively reviewed the records of all children presenting with a benign focal bladder mass during the last 5 years, including 2 girls and 3 boys 2 to 12 years old (mean age 7). RESULTS: Presenting symptomatology included irritative voiding symptoms, suprapubic pain and hematuria. All bladder masses were diagnosed by ultrasonography and all patients underwent transurethral resection. Three children who had an inflammatory bladder tumor with myofibroblastic features required open tumor excision with preservation of the bladder. In the 2 remaining children urine culture was positive preoperatively and the diagnosis was an inflammatory bladder tumor with eosinophilic infiltration. Transurethral resection of the mass was performed without partial cystectomy. CONCLUSIONS: Inflammatory myofibroblastic and eosinophilic tumors represent forms of focal cystitis with a tumefactive component. Differentiation from the malignant bladder neoplasms of childhood has important consequences for therapy. Management of these benign but aggressive inflammatory lesions involves local control by endoscopic or open surgery with bladder preservation. We encourage refined use of the terminology for inflammatory myofibroblastic and eosinophilic tumors.
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3/77. Aspiration cytology of the collagenized variant of mammary myofibroblastoma: a case report with review of the literature.

    Myofibroblastoma of the breast is a rare benign stromal neoplasm, which occurs primarily in men. Classical myofibroblastoma is a circumscribed, nonencapsulated tumor comprised of bipolar fusiform cells arranged randomly, or in fascicles alternating with broad collagenous bands. Additional histologic variants (the cellular, collagenized, infiltrative, and epitheloid types) have been described. Several case reports describe the cytopathologic features of the classical and cellular variants. We report on a 70-yr-old woman, who presented with a circumscribed mass in her left breast. Aspiration biopsy showed paucicellular smears with singly distributed atypical spindle-shaped cells and rare fragments of collagenized stroma, raising suspicion of a phyllodes tumor. Histologic examination revealed spindle-shaped cells distributed in a diffusely collagenized stroma. Some nuclear atypia was present. To the best of our knowledge, this is the first case reporting the cytologic features of the collagenized variant of myofibroblastoma. Although we believe a specific diagnosis of myofibroblastoma can be rendered in a male based on the typical cytologic and clinical findings in the classical type, the variant forms are difficult to classify accurately and require excision for a definitive diagnosis.
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4/77. Primary intrathoracic meningioma: histopathological, immunohistochemical and ultrastructural study of two cases.

    Meningiomas are common, usually benign slow-growing neoplasms of the central nervous system thought to arise from meningocytes capping arachnoid villi. Primary ectopic meningiomas are exceedingly rare extracranial and extraspinal tumors of controversial origin; they are usually limited to the head and neck region or to the paravertebral soft tissues. Only one mediastinal ectopic meningioma and few pulmonary ectopic meningiomas have been described in the literature until now. Because of their rarity and their intriguing pathogenesis, we report here a second case of primary mediastinal meningioma and an additional case of primary pulmonary meningioma. Their possible origin and differential diagnosis are discussed.
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5/77. Inflammatory myofibroblastic tumor with CNS involvement.

    BACKGROUND: Inflammatory myofibroblastic tumors (IMT) represent a spectrum of neoplasms that occur in the mesentery and retroperitoneum and less frequently in the mediastinum of children and young adults. Transformation into inflammatory fibrosarcoma and metastases are rare. CASE REPORT:We report the case of a 16-year-old patient with an inflammatory myofibroblastic tumor of the mesentery with mediastinal metastases. Partial remission was obtained by chemotherapy with ifosfamide, dactinomycine, and vincristine. Two months later, relapse with infiltration of the meninges developed, and the patient died. CONCLUSION:This case demonstrates unusual features of an IMT: presentation with metastases, excellent response to chemotherapy, dissemination to the CNS.
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6/77. An extraluminal leiomyosarcoma of the iliac vein without thrombosis--a case report.

    Vascular leiomyosarcoma (LMS)is a rare malignant tumor arising from the muscle cells of the media of the vessels. Vascular LMS is often diagnosed as a result of the clinical manifestations of impaired venous flow such as edema and phlebothrombosis. The authors present a case of an LMS in a 72-year-old woman. physical examination revealed a round mass deep in the left inguinal region close to the inguinal vessels, fixed and not pulsating. There was no sign of left lower-limb edema nor of articular impairment of the hip. Inguinal and distal pulses were normal. Results of laboratory analysis, including values for the oncologic markers, were normal. An ultrasound scan of the left inguinal fossa showed a 50-by-30 mm mass of mixed aspect that adhered to the left common iliac artery. An echo color Doppler showed conservative arterial flow. Thus, a thrombotic aneurysm of the left iliac artery was diagnosed. Chest x-ray showed no pathological findings. An abdominal computerized tomograph (CT) scan confirmed the location of the neoplasm and revealed a narrowing of the left iliac vein compressed behind the mass. The finding was interpreted as a colliquative lymph node. The patient underwent explorative laparotomy with midline incision. The finding was a neoplastic bilobed mass compressing and infiltrating the left iliac vein with no cleavage surface between the mass itself and the venous wall. An intraoperative frozen section revealed a necrotic mass with a cortex of ambiguous interpretation. Surgical procedure was then finished with no further venous resection. Histologic examination revealed the presence of leiomyosarcomatous tissue with nuclear pleomorphisms inside necrotic material limited by a thin fibrotic capsule. According to Coindre's classification the tumor was a G3. After six months the patient is fit and a CT scan showed no evidence of recurring disease.
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7/77. Inflammatory myofibroblastic tumor of the gastroesophageal junction in childhood.

    We report a unusual case of an inflammatory myofibroblastic tumor arising at the gastroesophageal junction in a 14-year-old girl. The bland histologic appearance with concurrent infiltration into adjacent structures made diagnostic interpretation difficult, but suggested a neoplastic process. A literature review was undertaken to address diagnostic and management issues raised in this case. Although the anatomic location was unusual, clinical, grass, histopathologic, and immunohistochemical data substantiated the diagnosis of inflammatory myofibroblastic tumor. The bland histologic appearance was consistent with the most widely accepted view of inflammatory myofibroblastic tumor as a low-grade neoplasm. Wide surgical excision was performed. This is considered the preferred treatment given the potential risk of recurrence and aggressive behavior, most frequently noted with extrapulmonary disease. Although inflammatory myofibroblastic tumor represents an heterologous spectrum of benign to malignant neoplastic proliferations, the prognosis is good in casts with benign histologic features.
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8/77. Myofibroblastoma of the male breast: a diagnostic problem on fine-needle aspiration cytology.

    Myofibroblastoma is an uncommon neoplasm of the male breast. Herein, we describe the cytologic features seen in the fine-needle aspirate of a 45-year-old man. The smears were cellular with intimate association of tumor cells with extracellular matrix material. The cells were spindle to polygonal and were without significant atypia. Numerous mast cells were observed. Nuclear grooving was present only occasionally, although this was conspicuous histologically. The presence of hyaline bands in between tumor cells, another interesting feature, was appreciated retrospectively. This neoplasm was initially misinterpreted as a malignant soft tissue tumor. awareness of the cytologic features coupled with mammography should prevent a misdiagnosis of this tumor.
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9/77. Fine needle aspiration cytology of intranodal myofibroblastoma. A case report.

    BACKGROUND: Intranodal myofibroblastoma is a rare, primitive, mesenchymal neoplasm of the lymph nodes first described in 1989. It behaves in a benign fashion and has a great predilection for the inguinal region. CASE REPORT: A 56-year-old man was referred for fine needle aspiration cytology of an inguinal lymph node. Smears were moderately cellular, with a predominant population of single, small spindle cells with no atypia. Most neoplastic cells were distributed as dissociated, single cells, with few groups. The cells showed metachromatic stromal material with a fibrillary quality. Nuclei were elongated, with pointed ends and occasional twisted forms. A remarkable finding on Papanicolaou-stained smears was hemosiderin granules. After a cytologic report of low grade spindle cell tumor, the node was excised, and a histologic and immunohistochemical diagnosis of intranodal myofibroblastoma was established. CONCLUSION: Intranodal myofibroblastoma should always be considered when aspirating solitary inguinal lymph nodes. The presence of a low grade spindle cell pattern of variably dissociated cells with hemosiderin granules should lead to immunocytochemical studies. Muscle-specific actin expression in the absence of S-100 protein and vascular markers permits a more specific diagnosis.
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10/77. Inflammatory myofibroblastic tumour of the liver in a child: CT and MR findings.

    Inflammatory myofibroblastic tumour of the liver is a rare disease in children and is characterized by an inflammatory mass of uncertain aetiology which simulates a true neoplasm, often resulting in a diagnostic dilemma. We report a pathologically proven case of inflammatory myofibroblastic tumour of the liver in a 14-month-old boy with jaundice. CT and MRI showed an ill-defined, homogeneously enhancing mass located in the hilar portion of the liver with biliary obstruction. He underwent percutaneous transhepatic biliary drainage to relieve obstructive jaundice, followed by lobectomy and, finally, liver transplantation.
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