Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pituitary carcinomas are very rare tumors, nearly always presenting as widely invasive masses, although the hallmark of these lesions is the finding of distant metastases. One third of reported cases are prolactin (PRL)-secreting tumors. We report the case of a fatal pituitary carcinoma evolving within 4 years from a PRL-secreting microadenoma. A 22-year-old woman presented because of galactorrhea. Evaluation of the patient disclosed slight hyperprolactinemia and magnetic resonance imaging (MRI) showed a 7-mm intrapituitary lesion, which responded to treatment with cabergoline. About 4 years after the first evaluation she developed sudden headache, ptosis, and diplopia in the right eye. MRI disclosed the growth of a large pituitary mass, invading the right cavernous sinus. Despite two trans-sphenoidal surgical procedures followed by gamma-knife radiosurgery, the patient showed rapid local progression of the tumor and the occurrence of new lung lesions, probably of metastatic nature. The patient died 7 months after the development of her first neurological symptoms because of tumor apoplexy and subsequent subarachnoid hemorrhage. This case represents the first documented rapid evolution from a microprolactinoma initially responding to dopamine agonists to a fatal pituitary carcinoma.
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PMID:Evolution of a prolactin-secreting pituitary microadenoma into a fatal carcinoma: a case report. 1791 59

Primary pituitary sarcoma in the absence of previous radiation therapy is very rare, only eight cases having been reported. We present the first reported case of sclerosing epithelioid fibrosarcoma of the pituitary, a 56-year-old woman presenting with diplopia and panhypopituitarism. Magnetic resonance imaging showed a 1.2 x 0.4 x 0.5-cm sellar mass invading the right cavernous sinus. Despite surgery and radiation therapy, our patient developed intracranial metastases and died 2 years after the initial diagnosis. Sclerosing epithelioid fibrosarcoma is an infrequently occurring, distinct variant of soft tissue fibrosarcoma. Its immunophenotype includes vimentin and occasionally CD-34, B cell lymphoma-2, S-100 protein, or keratin expression. Although initially thought to be a low-grade lesion, sclerosing epithelioid fibrosarcomas may also behave in a high-grade manner.
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PMID:Sclerosing epithelioid fibrosarcoma of the pituitary. 1809 72

Sellar paragangliomas are very rare lesions with only 11 previous cases described in the literature. We present a further case of sellar paraganglioma. The patient is a 17-year-old man who developed headache, visual blurring, and diplopia. MRI showed a sellar lesion. Trans-nasal trans-sphenoid biopsy showed features of paraganglioma. He was treated by Stereotactic radiotherapy. Four months after treatment he developed bone metastases which was palliated by radiation, zoledronic acid, and chemotherapy. This is the first case of sellar paraganglioma showing metastases to bone.
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PMID:A rare case of paraganglioma of the sella with bone metastases. 1832 Mar 26

The incidence of malignant cutaneous melanoma is increasing faster than any other cancer. Thus, it will become an increasingly common source of metastatic disease to the eye, lids, and orbit. Herein, we have performed a systematic review of previously published cases including patient characteristics, clinical presentation, diagnostic techniques, current treatments, and outcomes. At the time of ocular diagnosis, nearly all reported patients had a known history of cutaneous melanoma and synchronous non-ocular metastases. Several aspects help in differentiating the tumors from primary uveal melanomas such as the presence of symptoms, rapidly growing multifocal tumors, vitreous seeding, and histopathological findings. Intraocular metastases (uvea, vitreous, retina, and anterior-segment) are more common and occur in younger patients than extraocular metastases (eyelids, orbit, and extraocular muscles). Palliative radiation therapy is often used for intraocular disease. Orbital metastases from cutaneous melanoma commonly involve the extraocular muscles resulting in diplopia and exophthalmos. The mainstays of extraocular treatment are surgical resection and radiation therapy. Unfortunately, there are few good options for systemic treatment of diffusely metastatic melanoma. Therefore, patients with ocular metastasis should be managed to prevent loss of vision or loss of the eye, and to maximize their quality of life.
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PMID:Cutaneous malignant melanoma metastatic to the eye, lids, and orbit. 1850 Dec 66

Chordomas are uncommon tumours occuring from remnants of the notochord. They are mainly localized in the sacrococcygium, the spine and the skull base. We focused our study on skull base chordomas and reviewed 531 published cases and 6 cases diagnosed in our institution. The six cases diagnosed at our hospital are described with a special mention of a unique case presenting with meningitis. Clinical features, radiology, histopathology and survival with different treatments are reviewed. Skull base chordomas can occur at any age and are slightly more frequent in males. Patients complain most often of diplopia and headaches. Abducens nerve palsy is the commonest neurological finding. Radiological examination should include both CT-scan which shows extensive osteolysis, calcifications and contrast enhancement and MRI for topographical definition. Metastases rarely occur. Treatment remains controversial but combined surgery and radiotherapy is the best treatment with a 5 year survival rate of 75%.
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PMID:Skull base chordomas: presentation of six cases and review of the literature. 1863 20

The rate of symptomatic improvement of visual symptoms associated with hematogenous metastases to the sella and pituitary was evaluated retrospectively in seven patients (five men, two women; mean age, 52.3 years) with primarily visual symptoms (diplopia alone in three, diplopia with blurred vision in one, blurred vision alone in one, loss of peripheral vision in one, and unilateral complete blindness in one). Symptom duration ranged from 0.5 to 2 months. The primary diseases were non-small cell lung cancer in two patients, renal cell carcinoma in two patients, prostate cancer in two patients, and medullary thyroid carcinoma in one patient. All patients had widespread metastatic disease. Three patients had a suprasellar tumoral component. One patient had a clival extension, and one patient had extension into the cavernous sinus. All underwent trans-sphenoidal surgery to correct visual symptoms. Gross total resection was achieved in three patients. Subtotal resections and a partial resection were performed in three patients and one patient, respectively. Surgical blood loss averaged 282 mL. One patient died from sepsis. Five patients developed complications (cerebrospinal fluid leakage in three, diabetes insipidus in two, anterior pituitary dysfunction in two, and colitis in one). At a mean follow-up of 15 months, three patients were alive. Visual symptoms improved in five patients and were unchanged in two. Trans-sphenoidal surgery helped improve visual symptoms in most patients. The morbidity rate was high and likely related to the locally destructive and extensive nature of the lesions in overall morbid patients with widespread metastatic disease. Unless nonoperative measures can provide equal results, however, this approach provides reasonable palliation.
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PMID:Efficacy of Trans-septal Trans-sphenoidal Surgery in Correcting Visual Symptoms Caused by Hematogenous Metastases to the Sella and Pituitary Gland. 1876 52

A 46-year-old woman with known multiple endocrine neoplasia type 2A was referred for evaluation of left-sided progressive diplopia and proptosis. She was given the presumptive diagnosis of progressive thyroid-associated orbitopathy. Clinical examination revealed significant proptosis and restricted motility of the left eye. Serial CT demonstrated a marked progression in size of the left inferior rectus muscle. This was postulated to be a rare case of metastasis of a medullary thyroid carcinoma to the left inferior rectus muscle, which was confirmed by lateral orbitotomy and excisional biopsy. The presence of unilateral proptosis raises the possibility of intraorbital or intracranial disease and warrants further investigation. Detection of metastases and subsequent surgical excision or debulking has palliative benefits, including prevention of compression or invasion of major structures and cooperative planning with oncology and participation in treatment regimens that may prove beneficial in patients with metastatic medullary thyroid carcinoma.
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PMID:Orbital metastasis from medullary thyroid carcinoma. 1903 50

Thyroid cancers rarely metastasize to the brain: the incidence of brain metastasis of differential thyroid carcinomas has been reported at only 0.69%-1.3%. We report a case of a solitary brain metastasis from thyroid follicular carcinoma found 13 years after primary surgery. A 73-year-old woman was admitted to our hospital for investigation of headaches, double vision, and facial nerve palsy, 13 years after a subtotal thyroidectomy for thyroid follicular carcinoma. Magnetic resonance imaging (MRI) showed a solitary mass in the midbrain associated with a focal osteolytic lesion of the temporal bone. Further radiological examinations revealed no other mass lesion. After making a provisional preoperative diagnosis of a primary brain tumor, we performed subtotal removal of the tumor. Histopathological examination confirmed metastasis of thyroid follicular carcinoma. Thus, the patient underwent completion thyroidectomy followed by internal radiotherapy. She has remained well in the 4 years since her operation, without any signs of further recurrence.
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PMID:Solitary cranial metastasis of thyroid carcinoma 13 years after primary surgery: report of a case. 1913 67

Solitary extramedullary plasmacytomas are isolated plasma cell tumors of soft tissue that typically do not metastasize. They are rare and account for 4% of all plasma cell tumors. To our knowledge, only 14 cases of solitary extramedullary plasmacytomas in the sphenoid sinus have been reported. A 32-year-old man presented to our department with complaint of ocular pain in the right eyeball and diplopia. Physical and neurological examinations revealed intact and prompt direct and indirect light reflexes in both pupils and limitation of extraocular muscle movement seen with the lateral gaze of the right eyeball. Magnetic resonance imaging suggested the presence of mucocele or mycetoma, therefore surgical resection was performed with endoscopic endonasal transsphenoidal approach. Histopathology was consistent with plasmacytoma. Systemic work-up did not show any evidence of metastasis and the sphenoid sinus was the sole tumor site, and therefore the diagnosis of solitary extramedullary plasmacytoma was confirmed. We report a rare case of solitary extramedullary plasmacytoma in the sphenoid sinus with successful treatment using the endoscopic endonasal transsphenoidal resection and adjuvant radiotherapy.
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PMID:Endoscopic endonasal transsphenoidal resection of solitary extramedullary plasmacytoma in the sphenoid sinus with destruction of skull base. 1976 19

This 18-year-old woman presented with headache and diplopia over several months and was found to have an enhancing pineal tumor with resultant obstructive hydrocephalus. Following standard preoperative diagnostic tests, including spinal axis imaging, the patient was taken to the operating room for an endoscopic third ventriculostomy to relieve hydrocephalus and then subsequently underwent a craniotomy for gross-total resection of the pineal mass. The patient was discharged after an uneventful hospital course and received standard adjuvant cranial-spinal radiation and chemotherapy as an outpatient. Follow-up imaging 1 year after surgery demonstrated a metabolically active, lytic lesion in the C-3 vertebral body and new lung lesions suggesting a metastatic pineoblastoma. The patient underwent a C-3 anterior corpectomy and reconstruction without complication as aggressive therapy for presumed metastatic disease. Final pathological results from the vertebral lesion were consistent with radiation-induced reactive changes, not metastatic pineoblastoma as originally suspected. The patient recovered well and remains symptom free. To the authors' knowledge this is the first reported case of reactive changes mimicking metastasis in a single vertebral body following standard therapy for resected primary pineoblastoma.
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PMID:Post-radiation reactive changes in a single vertebral body mimicking metastatic pineoblastoma. 1987 84


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