Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuroblastoma, which is primarily an extracranial disease, has remote manifestations which can be confusing. Neurologic signs and symptoms such as opsoclonus (dancing eyes), ataxia, blindness, and cord paralysis may precede by several months the manifestations of the primary tumor in a distant site such as the abdomen. Descriptions of nine cases demonstrate the various modes of presentation and some of the unusual aspects of this disease. The recent advent of computed tomography requires a reevaluation of the neuroradiologic approach to the evaluation of neuroblastoma.
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PMID:Remote effects of neuroblastoma. 9 12

In 90 patients with known extra-hepatic malignancy the liver was examined for metastases. The diagnostic value of clinical information, blood examinations, 99mTc scintiscan, and laparoscopy for the diagnosis of the liver metastases was evaluated. Clinical data (age, sex, time since onset of symptoms and localisation of primary tumor) are of no diagnostic value. The most reliable blood tests are alkaline phosphatase (AP) and GOT. The probability of liver metastastases rises with increasingly abnormal values of AP and GOT. However, the probability is not much greater in cases with highly abnormal values than in cases with only moderate elevation of AP and GOT. Diagnostic accuracy of AP is optimal by using a cutoff point of 76 U/l (sensitivity 79%, specificity 64%). Bilirubin, prothrombin time, haemoglobin and blood sedimentation rate are of very little value. Combinations of AP with these blood tests does not improve diagnostic accuracy. Therefore, it is not useful to determine more blood tests than AP alone. Informed reading of liver scans has a specificity of 75% and a sensitivity of 91%. Blind reading of scans has a sensitivity of 94% and a specificity of 95%. This diagnostic accuracy cannot be improved by additional blood tests. Laparscopy has a sensitivity of 85% and a specificity of 95%. Scanning and laparoscopy are complementary methods. When optimal diagnostic accuracy is required both methods should be used.
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PMID:[Liver metastases: diagnostic value of blood tests, scintiscanning, and laparoscopy (author's transl)]. 13 43

Data from a series of 45 internal fixations for malignant secondary tumors of the humerus are analyzed. The tumors occurred late in the evolution of the cancer, and represented 24% of appendicular bone metastases for which operations were performed. Statistics of the patients, fractures, treatment, and results are presented and conclusions drawn. Prognosis should be related to the types of primary tumor. Blind nailing or pinning is recommended, and indications for preventive osteosynthesis are discussed.
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PMID:Surgical treatment of malignant secondary tumors of the humerus: report of 45 cases. 50 69

Primary tumors of the pineal body can produce dyscoordinative movements of the eye, pupillary dilatation, paralysis of adduction during convergence and nystagmus. Obstruction of the aqueduct can cause hydrocephalus, increased intracranial pressure and papilledema. Diabetes insipidus may be a presenting symptom. Pinealocytes and the photoreceptors of the eye contain several autoantigens. In man, the best known is the S-antigen. This antigen can be detected in the cerebrospinal fluid of patients with primary tumors of the pineal body. The S-antigen, and possibly other related autoantigens, can elicit an autoimmune mediated reaction causing inflammatory eye symptoms. This recently described paraneoplastic neurologic syndrome shares properties in common with other known cancer-associated ophthalmologic syndromes characterised by rapid development of eye symptoms, rapid loss of sight and by eye manifestations prior to evident appearance of symptoms related to primary tumor growth. A primary tumor of the pineal body should be considered in patients where a monosymptomatic uveoretinitis presents without associated provoking factors. Furthermore, analyses of S-antigen in the spinal fluid can be useful in the clinical diagnosis of the same primary tumors.
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PMID:[Physiopathologic mechanisms behind eye symptoms in primary tumors of the pineal body]. 843 Apr 65

A 2-year-old boy with blindness as an isolated symptom was found to have no light perception binocularly because of compression of both optic nerves by a neuroblastoma infiltrating the walls of the optic canals and medial sphenoid bone. Imaging disclosed a primary tumor near the kidney and multiple osseous metastases. Although neuroblastoma commonly causes blindness by metastasis to the orbit, it rarely causes bilateral blindness from intracranial compression of the optic nerves. This is the first report of bilateral blindness as the presenting feature.
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PMID:Metastatic neuroblastoma presenting with binocular blindness from intracranial compression of the optic nerves. 1517 64

Soft tissue sarcomas in children are a heterogeneous group of malignant diseases. Among these, tumors localized in the head and neck region are especially difficult to treat. While multidisciplinary care has dramatically improved the prognosis of sarcoma patients, their treatment remains uncertain because of demand on radical surgical resection of the tumor. Achieving cure without deforming or mutilating the child remains the primary goal of treatment. This study is the multicenter (nationwide, 11 Polish centers) retrospective analysis of the treatment results in children having soft tissue sarcoma in the head and neck region during the previous decade (from 1991 to 2001). Late effects of the treatment are documented in long-term survivals. Eighty-five children from 1 to 212 months of age were included. Different multimodal treatment protocols were utilized (CWS-91, SIOP-MMT-91, and CWS-96). The median observation time was 25 months. Data on long-term effects were collected in 34 long-term survivals. Complete remission was achieved in 68 (80%) patients. Primary treatment failure occurred in 13 (15.3%) patients, all of whom succumbed in disease progression. Relapse occurred in 21 (30.9%) patients primarily achieving complete remission. Second primary neoplasm occurred in 3 children. The estimated 5-year event-free survival and the 5-year total survival rates for the whole group are 0.38 and 0.55, respectively. The main late effect documented in long-term survivals were cosmetic defects in 12 (35.3%) and visual field deficit or blindness in 8 (26.5%). Despite substantial improvement of the prognosis of pediatric soft tissue sarcomas, the multimodal treatment of head and neck region tumors remains controversial. Improved long-term outcome and focusing on psychosocial difficulties raise the important and difficult problem of functional results and cosmesis. Tumors localized in the orbit carry an excellent prognosis. However, the main late sequela is vision impairment and cosmetic defect due to the therapy given many years earlier. Two other tumor localizations--the parameningeal and nonparameningeal ones--still have bad prognosis. The observations made in this study confirm that main prognostic factors are the size of the primary tumor and the tumor stage. The worst prognosis remains invasive tumor (T2-stage) with a size over 5 cm. Individually adjusted multimodal therapy, which imperatively needs to be radical, though not mutilating, might minimize the late effects. Psychosocial problems in long-term survivors need to be focused on at the national level and better support must be provided in the future, involving a team of different medical and paramedical profiles.
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PMID:Remaining problems and controversies in the management of childhood head and neck soft tissue sarcomas: Retrospective (national) Multicenter Study of the Polish Pediatric Solid Tumors Group. 1520 98

From 1989 to 2005, 28 patients--20 men and 8 women--with cervical lymph node metastasis from an unknown primary carcinoma were treated and studied retrospectively. In histological diagnosis, open biopsy was conducted in 11 patients and non-open biopsy (FNA or frozen section diagnosis during surgery) in 17. Blind biopsy under general anesthesia was conducted in 10 patients, showing one primary tumor in the nasopharynx. Tonsillectomy for diagnosis was not done. In region of maximum-size lymph node metastasis, the upper cervical region accounted for 22 cases (79%). The N stage of cervical lymph nodes was as follows: N2a in 4, N2b in 14, N2c in 3, and N3 in 7. The histopathological diagnosis of cervical lymph node was as follows: squamous cell carcinoma in 21, adenocarcinoma in 3, mucoepidermoid carcinoma in 2, and others in 2. Therapy was as follows: only neck dissection in 7, neck dissection with postoperative radiation therapy in 13, and irradiation and chemotherapy in 8. All patients treated with irradiation and chemotherapy had been judged to be inoperable. Seven patients were found to have a subsequent primary tumor. Primary tumor sites were as follows: tonsils in 3 and upper gingiva, base of tongue, lung, and nasopharynx in 1 each. FDG-PET was conducted in 7 patients but revealed no primary tumor. Overall 5-year survival in this study was 46%. We should pay particular attention to the tonsils for detecting primary tumors in patients with cervical metastasis from an unknown primary carcinoma.
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PMID:[Clinical study of 28 cases of cervical lymph node metastasis from an unknown primary carcinoma]. 1769 98

NHL usually presents with lymphadenopathy or symptoms related to compression by the primary tumor of surrounding structures. While the head and neck region is a common site of involvement, blindness is rarely a presenting symptom. We report here the case of a child who presented to the emergency room with acute bilateral loss of vision and no other symptoms. Cranial imaging studies revealed a solid mass of the skull base with compression on optic nerves. Diagnosis of Burkitt's lymphoma was confirmed after biopsy. The patient had partial vision improvement two days after optic nerve decompression which was done immediately at the night of presentation.
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PMID:Acute bilateral blindness as a presenting symptom of Non-Hodgkin's lymphoma. 2239 18

Intraocular choroidal metastasis is a very rare cause of blindness. Carcinoma of breast is the most common primary malignancy the accounts for choroidal metastasis in females. Other primary neoplasms which can uncommonly metastasize to the choroid are gastrointestinal tract, thyroid, pancreas, prostate and testis. Metastatic neoplasm to the eye outnumbers the primary tumors such as retinoblastoma and malignant melanoma. We present a case of sudden loss of vision due to breast cancer metastasis to the eyeball. The interval between the diagnosis of the primary tumor and the choroidal metastasis was 4 years.
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PMID:Sudden loss of vision due to breast cancer metastasis to the eyeball. 2503 5

Cancer-associated retinopathy (CAR) is a rare paraneoplastic disorder of the retina leading to blindness, associated with multiple cancers. It can lead to rapid progressive visual deterioration with retinal pathology ranging from retinitis pigmentosa to retinal degeneration. It is caused by antibodies directed against retinal antigens. This uncommon syndrome is a remote effect, independent of the primary tumor or metastatic lesion. We describe two cases of CAR, as well as pathophysiology, clinical manifestation, diagnostic criteria, and treatment of cancer-associated retinopathy.
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PMID:Case Series of Cancer-associated Retinopathy (CAR). 3141 17


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