Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Choriocarcinoma is the most malignant tumor of gestational trophoblastic neoplasia. It grows rapidly and metastasizes to the lung, liver, and, less frequently, to the brain. One rare case of metastatic cerebral choriocarcinoma with initial presentation of intracerebral hemorrhage is reported. A 40-year-old woman initially presented sudden onset of headache. Intracerebral hemorrhage resulting from ruptured pseudoaneurysm was suspected. Emergent surgery with excision of the pseudoaneurysms was performed. Metastatic choriocarcinoma was accidentally found with positive immunohistochemical staining of cytokeratin and beta subunit of human chorionic gonadotropin (beta-HCG). Choriocarcinoma with brain metastases was diagnosed. She then received chemotherapy with regimen of etoposide, methotrexate, actinomycin-D, cyclophosphamide, and vincristine (EMACO). Elevated serum beta-HCG (30.3 mIU/mL) and new pulmonary lesions were noted by computed tomography 4 months after completion of EMACO. Salvage chemotherapy with etoposide, methotrexate, actinomycin-D, etoposide, and cisplatin (EMAEP) regimen was given. Seven months later after completion of EMAEP, two new pulmonary lesions were detected by positron emission tomography (PET) scan. So she received video-assisted thoracoscopic surgery with tumor excision. Pathologic report confirmed the diagnosis of lung metastases. The patient recovered well. She is free of disease for 12 months. The diagnosis of metastatic cerebral choriocarcinoma was only made by histopathology after craniotomy. Metastatic choriocarcinoma should be always in the differential diagnosis for women at childbearing age presenting with unexplained stroke-like symptoms. In addition, PET scan may be valuable in detecting occult metastatic lesions of choriocarcinoma.
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PMID:Intracerebral hemorrhage as initial presentation of gestational choriocarcinoma: a case report and literature review. 1742 77

Primary central nervous system atypical teratoid/rhabdoid tumors mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumor, medulloblastoma and choroid plexus carcinoma, but present different biological behaviors. We present the case of an 18 year-old man who presented headache, vomiting and ataxia. CT-scan and MRI revealed a posterior fossa tumor. A gross total resection was performed. An intraoperative study showed papillary-like tumors with large cells and mitotic features. Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells with papillary features and rhabdoid cells. Immunohistochemically, these rhabdoid cells were positive for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, S-100 protein, and glial fibrillary acidic protein. Electro-microscopically, the typical rhabdoid cells contained whorled bundles of intermediate filaments in their cytoplasm. A rhabdoid tumor is a clinical-pathological entity and emphasizes the necessity to distinguish this unique tumor from other pediatric central nervous system neoplasms. Cytopathological features, immunohistochemistry and electro-microscopy differential diagnoses are discussed.
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PMID:Rhabdoid choroid plexus carcinoma: a rare histological type. 1787 20

Pituitary carcinomas are rare primary adenohypophyseal tumors with cerebrospinal or extracranial metastasis. The present case, the first report of the disease in Korea, involved a 36-yr-old woman who presented with a 3-week history of headache. Brain magnetic resonance imaging revealed a 2.5-cm sellar and suprasellar mass showing heterogeneous enhancement with suspicious invasion of both cavernous sinuses. The patient underwent gross-total resection. The tumor cells were composed of polygonal cells singly or in variable-sized nests. The nuclei were large and round with prominent nucleoli. The cytoplasms was acidophilic and granular. Marked pleomorphism and frequent mitoses (3 per 10 HPFs) were found. By immunohistochemistry, tumor cells were strongly positive for prolactin, but negative for ACTH and GH. Additional immunostainings for cytokeratin, vimentin, and glial fibrillary acidic protein (GFAP) were negative. After the surgery, the patient received radiotherapy because of the atypical histologic features. The prolactin level fell from 123.17 ng/mL to 5.17 ng/mL after surgery. Nine months after the initial diagnosis, the patient died from mandibular metastasis associated with the pituitary carcinoma.
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PMID:Pituitary carcinoma with mandibular metastasis: a case report. 1792 42

Choroidal masses are rarely the first presentation without the primary tumor being discovered. We described fine needle aspiration biopsy (FNAB) of a choroidal mass for diagnosis and determining the primary site. The patient, a 50-year-old Caucasian male without significant past medical history, presented with visual disturbances and headaches. Intraoperative ocular FNA was performed which was sparsely cellular showing a few loosely cohesive sheets and singly arranged epithelial cells with moderate amount cytoplasm, round large nuclei and prominent nucleoli. Immunohistochemical stainings on the cell block material showed positive staining of cytokeratin and negative staining of melanoma markers. The diagnosis of metastatic adenocarcinoma was rendered. During clinical follow up studies, the patient was found to have a PET positive lung nodule and multiple visceral metastasis.
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PMID:Choroidal metastasis from an occult primary diagnosed by fine-needle aspiration: a case report. 1897 16

Leptomeningeal (LM) dissemination arises rarely in patients with low-grade gliomas. Few cases of LM dissemination of spinal cord low-grade gliomas have been reported in children. Here, we report a case of an intramedullary spinal cord low-grade glioma occurring in an adult presenting by LM dissemination. A 57-year-old man presented with a history of headaches and visual acuity deterioration. Neurological examination revealed mental confusion, decreasing left limb strength and left positive Babinski sign. Lumbar puncture showed low CSF glucose, high CSF protein and no tumor cells. Spinal MRI revealed an intramedullary spinal lesion with a diffuse leptomeningeal enhancement. A spinal meningeal biopsy showed arachnoiditis with isolated atypical cells which were cytokeratin immunopositive. A differential diagnosis was proposed between arachnoiditis close to an infectious process or close to meningeal carcinomatosis. An intramedullary biopsy was programmed but the patient died 1 month after biopsy. The post-mortem examination showed thickened leptomeninges and an ill-circumscribed intramedullary grey lesion. Histological examination revealed a low-grade glial neoplasm composed of monomorphous spindle "piloid" cells with diffuse dissemination to leptomeninges. Glial fibrillary acidic protein immunostaining showed scattered immunoreactive cells. Clinical and pathological features of this case are unusual. Pathogenesis and prognosis of low-grade gliomas with LM dissemination are poorly understood. This case, like cases occurring in children, cannot easily be classified in the present WHO system of classification of CNS tumors. Spinal low-grade gliomas with LM dissemination may represent a possible distinctive clinico-pathological entity.
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PMID:Leptomeningeal dissemination of low-grade intramedullary gliomas: about one case and review. 1912 71

The nongerminomatous germ cell tumor occurring in the fourth ventricle is extremely rare. A 9-year-old boy presented with symptoms of obvious headache, projectile vomiting, diplopia and motor weakness. MRI scanning revealed lesions occupying the fourth ventricle, with dual-lateral ventricle expansion after gadolinium administration. Suboccipital craniectomy was performed and the tumor was removed by block resection. Twice intraoperative biopsy specimens revealed fiber vascular tissue and hemangioma. Histological examination revealed that the tumor consisted of loose network structures and Schiller-Duval bodies. Immunohistochemical study showed that the primitive germ cells were positive for placental alkaline phosphatase, alpha-fetoprotein, cytokeratin, vimentin, but negative for glial fibrillary acidic protein. The histological diagnosis was pure yolk sac tumor. Two years after surgery, he has suffered no tumor recurrence. A combination of surgical treatment, radiation and chemotherapy is mandatory.
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PMID:Yolk sac tumor in the fourth ventricle: a case report. 1954 38

We report a case of malignant solitary fibrous tumor involving the pineal region in a 49-year-old woman. The patient presented with headache, slowly progressive weakness of the right lower extremities and upgaze palsy over the past year. Histologically, the tumor was composed of moderately hypercellular proliferated spindle cells with eosinophilic collagen bands. These cells were diffusely and strongly immunoreactive with CD34, CD99, and vimentin, but were negative with epithelial membrane antigen, S-100 protein, Bcl-2, smooth muscle actin, cytokeratin and glial fibrillary antigenic protein. MIB-1 labeling indices and mitosis rates were 7.3 +/- 1.8% and 5 per 10 high power fields, respectively. Ultrastructural examination revealed that the neoplastic cells had features of fibroblastic differentiation. Differential diagnoses included fibrous meningioma and hemangiopericytoma. The present case provides one unique example of a rare entity to the already diverse spectrum of the pineal region neoplasms encountered in neuropathology.
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PMID:Malignant solitary fibrous tumor arising from the pineal region: case study and literature review. 1984 65

The author reports herein a case of small cell carcinoma of the brain without extracranial tumors by serial imaging modalities. A 75-year-old man presented with headache. Brain CT and MRI revealed a solitary cystic tumor (5 x 6 x 7 cm) in the left occipital lobe. Blood laboratory test revealed no significant findings. Preoperative diagnosis was a primary or metastatic brain tumor. Preoperative systemic examinations including CT, MRI and PET revealed no extracranial tumors. Tumorectomy was performed. Pathologically, the tumor was small cell carcinoma positive for four types of pancytokeratins, cytokeratin (CK) 7, CK 18, thyroid transcriptional factor-1 (TTF-1), CD56, chromogranin, synaptophysin, neuron-specific enolase, p53 protein, KIT, PDGFRA, and Ki-67 antigen (labeling = 100%). It was negative for high molecular weight CK, CK5/6, CK14, CK19, CK20, PE10, epithelial membrane antigen, vimentin, CEA, desmin, S100 protein, CA19-9, alpha-smooth muscle actin, CD34, p63, and CD68. The pathologic examination strongly suggested primary small cell lung carcinoma. However, repeated serial imaging modalities including systemic CT, MRI and PET revealed no extracranial tumors. The serial sputum cytology was always negative. The patient was treated with radiation and cisplatin-based chemotherapy, and no tumors were found seven months after the operation. The present case suggests that there are small cell carcinomas with a solitary brain metastasis without a radiologically detected primary site. In the present case, primary small cell brain carcinoma cannot be excluded completely, although such a case has not been reported in the literature.
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PMID:Small cell carcinoma of the brain without extracranial involvement by serial CT, MRI and PET. 2022 32

Papillary tumor of the pineal region (PTPR) is a recently recognized and rare pineal tumor, presenting as a solitary mass with or without hydrocephalus. Here, we report a case of c-Kit expressing PTPR with leptomeningeal seeding. A 39-year-old woman presented with a 1-month history of headache and decreased visual acuity. MRI showed a large, 4 cm-diameter solid and cystic enhancing mass at the pineal region with associated ventriculomegaly. Smaller nodular lesions were also found at the pituitary stalk and bilateral internal acoustic canal (IAC). The leptomeninges were noted to be enhanced with gadolinium. Endoscopic third ventriculostomy and partial resection were performed. The specimen was small in quantity but nonetheless, revealed the typical features of PTPR, which were tumor cells with vacuolated cytoplasm forming a pseudopapillary architecture. The tumor cells were diffusely immunoreactive for vimentin, INI-1 and c-Kit, focally immunoreactive for neuronal specific enolase (NSE) and S100 protein but negative for cytokeratin, epithelial membrane antigen (EMA), synaptophysin and GFAP. Ultrastructurally, the tumor cells revealed variably-sized cytoplasmic vacuoles, intermediate filaments and villous cytoplasmic membrane. With these features, a diagnosis of PTPR was rendered. The lesions at the pineal gland and bilateral IAC were irradiated through gamma knife radiosurgery and a decrease in size of the lesions was noted on follow-up MRI. However, soon after, other lesions were also noted to develop along the adjacent sites. The case presented is proof that PTPR can disseminate to other sites distant from the original lesion. This case was a c-kit expressing PTPR, which might represent the more primitive nature of this tumor. Ultrastructural examination is useful to differentiate PTPR from other tumors of the pineal gland in addition to immunohistochemistry.
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PMID:Papillary tumor of pineal region presenting with leptomeningeal seeding. 2037 98

Spindle cell oncocytoma (SCO) of the pituitary gland is a relatively recently established, very rare subtype of adenohypophysis tumours that was introduced as a distinct clinicopathological entity in the fourth edition of WHO classification of the central nervous system tumours (2007). It is non-endocrine neoplasm of the anterior pituitary that occurs in adults and usually follows a benign clinical course, corresponding to WHO grade I. Up to now, pituitary SCO have been reported occasionally and only 14 cases of SCO have been documented in the literature. Because of their rarity, the pathogenesis and natural history of these tumours have not been fully characterized. We report two additional cases of SCO occurring in females aged 63 years (Case 1) and 65 years (Case 2), who presented with pan-hypopituitarism, headache and visual field defect. In both cases, the magnetic resonance imaging showed solid sellar mass of moderate size with suprasellar extension. The clinical and radiological features suggested non-functioning pituitary macroadenomas without evidence of invasive growth. One patient presented with tumour recurrence 3 years after undergoing the previous surgical removal of tumour, which was initially misdiagnosed as schwannoma. The first tumour was removed by transsphenoidal surgery and the second one by frontal craniotomy. Histologically and immunohistochemically, both tumours displayed the features typical for SCO of the pituitary. They were composed of interwoven fascicles of spindle cells exhibiting abundant eosinophilic cytoplasm of oncocytic or granular appearance. Mitoses were rarely observed and necrosis was absent. In one case, the advanced lymphocytic infliltration was observed within neoplastic tissue. The tumour cells exhibited immunoreactivity for S-100 protein, galectin-3, vimentin and epithelial membrane antigen but they were negative for GFAP, anterior pituitary neuroendocrine markers (prolactin, growth hormone, TSH, ACTH, FSH, LH), chromogranin, synaptophysin, cytokeratin CK (AE1/AE3), smooth muscle actin, desmin, CD34 and CD68. MIB1 labeling index did not exceed 10%. Ultrastructurally, the tumour cells were rich in mitochondria with lamellar cristae. Moreover, in Case 2 some tumour cells showed a number of giant mitochondria with severely destructed internal matrix. Spindle cell oncocytoma of the anterior pituitary is often misdiagnosed entity of uncertain histogenesis. It should be considered in the differential diagnosis of various sellar-region lesions of oncocytic morphology.
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PMID:Spindle cell oncocytoma of the adenohypophysis - a clinicopathological and ultrastructural study of two cases. 2092 1


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