Cases reported "Abdominal Neoplasms"

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1/108. Autonomic nerve tumour with skeinoid fibres: ultrastructure of skeinoid fibres examined by quick-freezing and deep-etching method.

    A case of gastrointestinal autonomic nerve tumour with skeinoid fibres (SFs) of the jejunum in a 79-year-old Japanese man, was examined by the quick-freezing and deep-etching (QF-DE) method. The tumour consisted of spindle cells with immunohistochemical reactions for vimentin, NSE and CD34. Electron microscopically, features of the neural cells of the myenteric plexus were observed. The QF-DE method demonstrated intercellular meshwork structures, consisting of thin filaments (7-15 nm), with granular deposits. Fully developed parts of the deposits formed nodular aggregates composed of irregularly surfaced thick fibrils (30-48 nm) with a tendency to linear arrangement (SFs). We detected many interconnecting thin filaments (ICTFs) between the SFs, which were pre-existing components in the meshwork, avoiding the granular deposits. The focal thickening formed by the connection between SFs and ICTFs revealed a periodicity typical of SFs (33-45 nm). We conclude that SFs are formed by decoration of the granular deposits along pre-existing intercellular meshwork structures.
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2/108. Squamous cell carcinoma of suprapubic cystostomy tract without bladder involvement.

    This report describes a third case of squamous cell carcinoma of the suprapubic cystostomy tract. The first case reported in 1993 concerned a squamous cell carcinoma arising adjacent to the suprapubic cystostomy site and extending anteriorly to the abdominal wall in a 80-year-old man, 5 years after suprapubic urinary diversion for urethral stricture. A second case published in 1995 described a 50-year-old paraplegic man (T11-T12 spinal cord injury) in whom a suprapubic cystostomy tract squamous cell carcinoma developed after 25 years of urinary diversion. The tumour involved the cystostomy tract primarily with extension into the bladder but did not penetrate the bladder wall muscle. Our patient is in fact the second one to have a suprapubic cystostomy tract squamous carcinoma not involving the bladder.
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3/108. Surgical aspects of iliopsoas compartment tumours.

    AIM: To demonstrate the pathological variety and highlight the surgical principles involved in the management of tumours of the iliopsoas compartment (IPC). methods: review of four clinical cases. RESULTS: Adequate surgical resection or palliation was achieved in each case. CONCLUSION: Resection of IPC tumours is feasible but access to the most superior part of the muscle may be impossible. An extraperitoneal approach is advocated.
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4/108. Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature.

    Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.
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5/108. Squamous cell carcinoma of the cervix simulating an advanced malignancy of the ovaries.

    A 40-year-old woman was diagnosed as having stage II squamous cell cervical carcinoma and managed with radiotherapy. Three months after treatment, she presented with features suggestive of an advanced ovarian tumour including gross abdominal swelling, bilateral ovarian tumours, multiple tumour seedlings in the abdominal cavity and ascites. There was also pleural effusion. Operative findings revealed widespread intra-abdominal metastases whose histology, contrary to expectations, showed squamous cell carcinoma of cervical origin. Distant metastases from squamous cell carcinoma of the cervix are rare. A high index of suspicion is necessary to detect this unusual mode of presentation.
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6/108. Abdominal desmoid masses in Gardner's syndrome.

    Abdominal masses, "desmoid tumors," occur in approximately 4% of patients with Gardner's syndrome, usually 1--3 years after total colectomy. Histologic sections usually resemble those of an infiltrating fibrous tumour, a desmoid lesion. The radiographic pattern may be similar to any diffuse abdominal retroperitoneal tumor, metastatic disease, lymphoma, or possibly retractile mesenteritis. The clinical setting usually differentiates these entities.
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7/108. Rare presentation of small bowel leiomyosarcoma with liver metastases.

    Intraabdominal sarcomas are rare tumours usually diagnosed at an advanced stage. These lesions at presentation are bulky and symptoms are often related to pressure effects on adjacent organs. This case report describes a rare presentation of a small bowel leiomyosarcoma whose initial presentation was free haemorrhage into the abdominal cavity and concomitant liver metastases. This case report also demonstrates that, even with such a rare presentation, an aggressive surgical approach is indicated in this type of tumour and helps a patient with advanced disease to live a few disease-free months with a good quality of life.
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8/108. Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma.

    neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.
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9/108. Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure.

    A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.
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10/108. Massive abdominal and pelvic myxoma in Carney's syndrome.

    This report describes a massive abdominal and pelvic myxoma in a patient with Carney's syndrome. A 38 year old woman presented with abdominal distension and a palpable mass, and at operation a large pelvic and abdominal tumour was identified and resected. The surgical specimen consisted of a lobulated mass, which on cut section had a uniform gelatinous consistency. The mass surrounded both ovaries, the appendix, and the upper part of the uterus, but macroscopically did not appear to involve these organs. Histological examination showed plump stellate and spindle shaped cells set in an abundant myxoid stroma, in keeping with a myxoma. Immunohistochemical staining revealed positivity of tumour cells for vimentin, but no reactivity to desmin, alpha-smooth muscle actin, S-100 protein, CD34, or AE1/AE3. This is the first documented case of massive adominal and pelvic myxoma in a patient with Carney's syndrome. Clinicians and pathologists should be aware that myxomas in Carney's syndrome can rarely involve unusual sites other than the skin and heart.
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