Cases reported "Disease Progression"

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1/86. Digital clubbing in a patient with progressive mantle cell lymphoma.

    Digital clubbing with or without hypertrophic osteoarthropathy may occur in a variety of neoplastic diseases. We present the first description of digital clubbing in a patient with progressive mantle cell lymphoma, in the absence of any other apparent etiology. We suggest that clubbing might be a paraneoplastic manifestation in mantle cell lymphoma. The literature on digital clubbing in hematological neoplasms is reviewed.
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2/86. Prolonged remission of recurrent, metastatic placental site trophoblastic tumor after chemotherapy.

    BACKGROUND: Placental site trophoblastic tumor (PSTT) is a form of gestational trophoblastic neoplasm that is frequently resistant to chemotherapy. In most cases disease is confined to the uterus and can be cured by curettage or simple hysterectomy. patients with metastases, however, frequently have progression of disease and die despite aggressive multiagent chemotherapy. CASE: A 31-year-old woman was found on review of uterine curettings to have a PSTT. Imaging studies revealed multiple lung lesions, a liver lesion, and an enlarged irregular uterus. hysterectomy and staging surgery revealed a large tumor in the endometrial cavity and multiple metastases. She was treated with etoposide-methotrexate-dactinomycin and cyclophosphamide-vincristine and had a complete clinical remission. Six months later, however, she had a recurrence. She was then treated with six cycles of etoposide-methotrexate-dactinomycin and etoposide-cisplatin. Three years after completion of the second regimen she is without evidence of disease. CONCLUSION: Treatment with multiagent chemotherapy can produce long-term remission, even in patients with recurrent, metastatic PSTT. Addition of platinum may be helpful in patients who have recurred or progressed after treatment with non-platinum-containing regimens.
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3/86. Radiographic manifestation of clear cell odontogenic tumor.

    Clear cell odontogenic tumors are uncommon, only 20 cases having been reported in the literature. We report a case that presented with unique radiographic manifestations over a period of 12 years; no similar case has been reported to date. documentation of the malignant nature of the condition and of the range of clinical and radiographic manifestations with which this neoplasm can present provides useful insight into its pathogenesis and progression. Early and timely recognition of the lesion, histopathologic examination, and aggressive interventional procedures are in order to successfully treat this condition and prevent a potentially fatal outcome.
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keywords = neoplasm
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4/86. Intracystic hemorrhage of pancreatic serous cystadenoma after renal transplantation: report of a case.

    Immunosuppressive therapy after transplantation increases the risk of developing neoplasms, and neoplasms of the digestive organs are very common in asia. We experienced a patient with an intracystic hemorrhage of pancreatic serous cystadenoma during the follow-up after renal transplantation. Pancreatic cystadenomas are not frequent. Only two cases, presenting with acute abdomen, have so far been reported in the literature. The intracystic hemorrhage in our case may have been related to a rapid tumor growth due to weakened antitumor immunity and azathioprine-induced pancreatitis.
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keywords = neoplasm
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5/86. Rapid progression of primary vaginal squamous cell carcinoma in a young hiv-infected woman.

    BACKGROUND: The association of human immunodeficiency virus (hiv) infection with rapid progression of cervical and anal squamous cell carcinoma has been clearly established by several studies. Human papilloma virus (HPV) infection of the anogenital tract is believed to be the causative agent of cervical, anal, vaginal, and vulvar squamous cell carcinoma. While a myriad of reports exist in the literature pertaining to the rapid progression of cervical and anal carcinoma in hiv-infected patients, no association of hiv infection and vaginal carcinoma has been reported. We present an unusual case of a young woman infected with hiv who was diagnosed with advanced vaginal carcinoma and succumbed to her disease shortly thereafter despite aggressive treatment. CASE: A 40-year-old woman with a 2-year history of hiv infection presented with Stage IVA squamous cell carcinoma of the vagina and a large vesicovaginal fistula from the tumor eroding through the posterior bladder wall. Computed tomography (CT) of the abdomen and pelvis revealed a large tumor replacing the vagina with mild hydronephrosis and diffuse pelvic and inguinal lymphadenopathy. She underwent urinary diversion with a transverse colon conduit followed by pelvic radiation with weekly cisplatin chemosensitization. A repeat CT scan of the abdomen and pelvis upon completion of her treatment revealed progression of disease with multiple liver metastases and gastrohepatic ligament adenopathy. She subsequently died of advanced metastatic vaginal carcinoma 2 months after completion of treatment. CONCLUSION: Due to the rarity of primary vaginal carcinoma, the clinical behavior of this neoplasm in the hiv-infected patient is poorly understood. Our case indicates that, although vaginal carcinoma is a disease of the elderly, young women infected with hiv and HPV are predisposed not only to develop cervical or anal carcinoma but also may be at increased risk for vaginal carcinoma with more aggressive and less responsive disease. Furthermore, although vaginal carcinoma is usually a slow-growing neoplasm, this case illustrates the aggressive behavior of such a tumor when associated with hiv infection.
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ranking = 2
keywords = neoplasm
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6/86. Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature.

    hyponatremia is a frequent event in neurosurgery practice and is usually associated with subarachnoid hemorrhage, head trauma, infections and neoplasms. The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation. In contrast to the better-recognized SIADH, there has not been a uniform consensus over the humoral and neural mechanisms of CSWS and functional aspects of renal response. In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.
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7/86. Microvascular endothelial abnormality in skeletal muscle from a patient with gastric cancer without dermatomyositis.

    We found a microvascular endothelial abnormality in a biopsy specimen from the gastrocnemius muscle of a patient with gastric cancer, who had severe myalgia and angialgia in the calf region with the symptoms of thrombophlebitis. There were no definite findings of inflammatory myopathy in histochemical and immunohistochemical studies. Electron microscopic examination revealed the accumulation of abnormal mitochondria in the subsarcolemmal area, and a fair number of degenerating capillaries. Immunohistochemical analysis of procoagulant or anticoagulant factors revealed marked reduction of thrombomodulin (TM) expression on small vessels and capillaries. Although a reduction of TM on small vessels has been observed around perifascicular atrophic fibers in patients with dermatomyositis, histochemical findings of the present patient showed no perifascicular atrophy or severely degenerating fibers. These pathological findings in the patient may be related to a malignant neoplasm and may be one of the causes of disseminated intravascular coagulation (DIC), which is the main complication of malignant neoplasms. Further studies are necessary to determine whether the reduction of TM on the small vessels and capillaries in skeletal muscle is a predictor of some severe condition such as DIC or a rare pathological finding in some special condition such as scirrhous carcinoma with thrombophlebitis.
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keywords = neoplasm
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8/86. Atypical features prompting neuroimaging in acute optic neuropathy in adults.

    BACKGROUND: Acute optic neuropathy due to an intracranial lesion may masquerade as optic neuritis or nonarteritic anterior ischemic optic neuropathy (NAION). We reviewed the records of patients who presented with acute unilateral optic neuropathy that was initially diagnosed as optic neuritis or NAION but who ultimately proved to have an underlying structural lesion. methods: Retrospective observational case series. We reviewed the records of patients with the initial diagnosis of optic neuritis or NAION in whom the diagnosis was changed to an intracranial etiology at four tertiary care neuro-ophthalmology centres between 1995 and 1998. RESULTS: Eight cases were identified in which atypical features prompted further investigation, including neuroimaging, leading to the diagnosis of an intracranial etiology for the optic neuropathy. Five patients were discovered to have neoplasms (a tuberculum sellae meningioma in two cases, an optic nerve sheath meningioma in two cases and a metastatic lesion in one case), and three patients had intracranial sarcoidosis. Atypical features for optic neuritis included a progressive course, absence of pain, optic atrophy at presentation, lack of significant visual improvement and age over 40 years. For NAION, the atypical features included progressive course, optic atrophy on presentation, absence of vasculopathic risk factors and preceding transient visual loss. INTERPRETATION: Clinicians should be aware that patients with intracranial lesions may present with acute optic neuropathy mimicking optic neuritis or NAION and that certain atypical features should warrant consideration for neuroimaging.
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9/86. Juvenile neutrophilic eccrine hidradenitis: a vasculitis-like plantar dermatosis.

    Neutrophilic eccrine hidradenitis is a variety of neutrophilic dermatosis described in patients with different neoplasms, most often leukaemia, and different chemotherapy regimens. It is characterised by neutrophilic infiltration of the eccrine coils of sweat glands. Recently it has been described in healthy juveniles, involving primarily the soles of the feet. We describe five new cases of juvenile neutrophilic eccrine hidradenitis all showing a good prognosis or a self-limiting course.
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keywords = neoplasm
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10/86. T-large granular lymphocyte leukaemia with natural killer cell-like cytotoxicity and expression of two different alpha- and beta-T-cell receptor chains.

    We describe a case of cytotoxic T-large granular lymphocyte leukaemia showing typical morphological features, expressing antigens characteristic for cytotoxic T cells and exhibiting marked natural killer-like cytotoxicity towards different target cells. Moreover, characterization of the T-cell receptors revealed simultaneous expression of two different types of beta-chains as well as alpha-chains by the malignant cell clone. The patient had an 8 year history of indolent disease, before progressing to an aggressive clinical course hardly responsive to chemotherapeutic treatment. This is the first description of a peripheral T-cell neoplasm expressing four distinct types of T-cell receptor molecules.
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