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)

Anorectal melanoma is a rare disease (1% of all anorectal malignancies). It is characterised by aspecific symptoms and the differential diagnosis versus other lesions of the rectum and anus is often difficult. The prognosis is very poor: mean survival is about 24 months, and at diagnosis most patients present distant metastases. Surgery is suggested as being the best treatment for this disease, since radio- and chemotherapy are generally only used for palliative purposes. Long-term survival depends on the stage of the melanoma at diagnosis. The possible surgical treatments available consist in local resection, which is considered the first therapeutic choice, and abdominoperineal amputation when local resection cannot be performed, or as a palliative operation. Inguinal lymphadenectomy is indicated when the inguinal lymph nodes are involved. In this report we describe a case of anorectal melanoma in a 73-year-old woman who underwent abdominoperineal amputation as surgical palliative treatment, because of infiltration of the puborectal muscle. The case report is followed by a review of the literature.
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PMID:[Primary ano-rectal melanoma: considerations on a clinical case and review of the literature]. 1293 6

Vulvar cancer is a rare disease. Squamous-cell carcinomas account for 90% of vulvar cancers. The main mode of spread is lymphogenic to the inguinofemoral lymph nodes. Therefore, elective uni- or bilateral inguinofemoral lymphadenectomy is part of the standard treatment in combination with radical (wide) local excision of the vulvar tumour. Lymph drainage studies in relation to the biological behaviour of vulvar cancer are presented, as well as the anatomy and surgery of the groin. The sentinel lymph node procedure is a relatively new method of staging in vulvar cancer which may lead to the omission of inguinofemoral lymphadenectomy in those patients identified as not having inguinofemoral lymph node metastases. The accuracy of this technique appears to be high, but its safety still has to be proven. Moreover, the role of additional histopathological techniques for the examination of the sentinel lymph nodes needs to be established.
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PMID:Groin surgery and the sentinel lymph node. 1296 33

Small bowel adenocarcinoma (SBA) is a relatively rare disease. Because of its rarity the role of chemotherapy either as adjuvant or for advanced disease has not been clearly defined. Therefore any information, including case reports, is warranted. We report on three patients with adenocarcinoma of the jejunum and ileum. Two patients with positive lymph nodes received postoperative adjuvant chemotherapy with 5-fluorouracil-folinic acid (5FU-FA) for 12 months but they developed metastatic disease 3 and 8 months later, respectively. The third patient was initially treated with the same agents but for metastatic disease. All patients were subsequently treated for tumor recurrence with irinotecan 350 mg/m2 i.v. every 3 weeks as salvage chemotherapy supported by Granulocyte Colony Stimulating Factor (GCSF) for 5 days. Two patients achieved a minor response and had a dramatic improvement of their symptoms. Their survival times after irinotecan administration were 14 and 6 months with an overall survival after primary diagnosis of 29 and 27 months, respectively. The third patient who had a tumor refractory to 5FU-FA progressed also on irinotecan and had an 8-month overall survival. Although conclusions cannot be drawn regarding the role of adjuvant chemotherapy in SBA, it seems reasonable to extrapolate from large bowel carcinoma experience. Irinotecan seems to have some degree of activity in the treatment of SBA but further studies are warranted.
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PMID:Irinotecan as salvage chemotherapy for advanced small bowel adenocarcinoma: a series of three patients. 1459 44

Three patients, a woman aged 46 years and two men aged 81 and 62 years, presented with abdominal pain, nausea, vomiting and/or weight loss. A small intestine follow-through series revealed a significant stenosis in all 3 patients. A laparotomic partial resection of the affected jejunum and corresponding mesentery was performed. A primary adenocarcinoma of the small intestine was diagnosed; pathology revealed that the resections were radical, and pT3N0, pT2N0 and pT3N0 stage tumours respectively. The first patient underwent a repeat operation four months later due to similar complaints caused by a tumour recurrence; fifteen months later she died from recurrent disease. The second patient was disease-free 3 years after surgery. In the third patient, liver and peritoneal metastases developed 16 months after surgery; he died 10 months after palliative chemotherapy had been initiated. Adenocarcinoma of the small intestine is a rare disease and patients often present late with aspecific complaints. This, combined with the fact that these tumours tend to follow an aggressive course, results in a poor five-year survival rate of 10-35%. Surgery is the only curative treatment currently available. A greater awareness of this type of tumour is needed for treatment results to improve.
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PMID:[Primary adenocarcinoma of the small intestine]. 1512 73

Zollinger-Ellison syndrome is characterised by refractory peptic ulcer disease, severe diarrhoea and gastric acid hypersecretion associated with an islet-cell tumour of the pancreas (gastrinoma). The true incidence and prevalence of this rare disease is unknown; in the US, the frequency is one per one million people and the age at presentation varies from 7 to 90 years. Zollinger-Ellison syndrome is sporadic in 62-80% of cases and in 20-38% of cases is associated with multiple endocrine neoplasia type 1 (MEN 1). The diagnosis of Zollinger-Ellison syndrome is certain when the plasma gastrin is >1000 pg/mL and the basal acid output is >15 mEq/h in patients with an intact stomach, >5 mEq/h in gastrectomised patients, or when this hypergastrinemia is associated with a pH <2. The treatment is based on control of gastric acid hypersecretion and of the malignant tumour and its possible metastases. Proton pump inhibitors are the most effective antisecretory drugs and can be administered in the elderly at high dosages without drug-related adverse effects. As an initial therapy, daily dosages of omeprazole 80-100 mg or pantoprazole 40-160 mg are employed. In long-term treatment the doses can be greatly reduced once effective control of the gastric output has been established. Intravenous proton pump inhibitors may be administered when patients cannot take oral therapy, particularly in acute conditions. All sporadic localised gastrinomas should be excised if possible. When liver metastases are also present, their debulking may improve symptoms and survival, and facilitate medical treatment. There is some controversy as to the surgical approach for gastrinomas associated with MEN 1. Somatostatin analogues can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric enterochromaffin-like (ECL) cells and can thus contribute to treating the disease more effectively. Their antiproliferative effect can be used in treating liver metastases. Chemotherapy is not the therapy of choice in patients with gastrinomas and is indicated only in those with malignant progressive disease; interferon alpha, embolisation and chemoembolisation are not advisable for the elderly. The treatment of elderly Zollinger-Ellison syndrome patients, similarly to all elderly oncological patients, should be based on the use of comprehensive geriatric assessment. This will enable the clinician to define the functional status of the elderly person, to decide whether the patient can tolerate surgery and/or the stress of antineoplastic therapy, and finally, to determine whether this patient can tolerate an aggressive treatment for Zollinger-Ellison syndrome or whether the only possible choice is palliative relief of symptoms.
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PMID:Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. 1465 42

Glomangiomas are a subset of glomus tumors that have a rich vascular network. Although a majority of the glomus tumors occur in the skin of the hand, they have also been reported in the deep soft tissue, bone, lungs, and gastrointestinal tract, especially the stomach. To our knowledge, only one such case has previously been reported primarily occurring in the liver. We report a case of a glomangioma primarily arising in the liver of a 57-year-old man who presented with right flank pain of several months' duration. A 3.0-cm hepatic mass was excised and consisted of numerous, small-to-medium branched vessels with the stroma containing small, round, regular cells with sharply outlined round-to-oval nuclei. Immunostains showed the tumor cells to be diffusely positive for vimentin and smooth muscle actin and to be focally positive for calponin. Collagen IV stained the pericellular matrix. The immunostain for CD34 highlighted the vascular network as well as outlined the tumor cells in many areas. Coexpression of actin and CD34 in glomus tumors, although unusual, has recently been reported in the literature. Despite its bland histology, the large tumor size and deep visceral location were suggestive of aggressive behavior; thus, a close clinical follow-up was recommended. The patient had an unremarkable postoperative course and has no evidence of metastatic disease 12 months after the procedure. An accurate diagnosis and an understanding the biology of this rare disease, especially in an unusual location, are crucial to its management.
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PMID:Primary glomangioma of the liver: a case report and review of the literature. 1498 33

Ovarian malignant melanoma (MM), primary or metastatic, is an extremely rare tumor and in the absence of a previous diagnosis can represent a diagnostic challenge. We present the clinicopathologic and immunohistochemical features of 23 cases seen in our institution over a period of 40 years (1962-2001). The patients' age ranged from 14 to 53 years (mean 35.7 years). Ethnicity was known in 19 patients: 14 white, 4 Hispanic, and 1 black. A previous history of MM was definitively obtained in 14 patients; in these cases, the interval between the primary MM and the ovarian metastasis ranged from 15 to 228 months (mean 77.7 months). The tumor was unilateral in 19 and bilateral in 4 cases. The tumor size ranged from 4.5 to 23 cm (average 10 cm); the melanoma arising in a cystic teratoma was 0.2 mm in thickness. The tumor was grossly pigmented in 8 cases (35%). The architectural pattern was nodular (8 cases), diffuse (6 cases), nodular and diffuse (5 cases), nested (3 cases), and lentiginous arising in a teratoma (1 case). Follicle-like spaces were seen in 8 cases, pseudo-glandular areas in 1 case, pseudo-myxoid areas in 1 case, and cords in 1 case. The tumor cell type was epithelioid in 19 cases, spindled in 2 cases, mixed epithelioid and spindled in 1 case, and small cell in 1 case. Nucleoli were prominent in 18 cases, and nuclear inclusions were present but rare in the majority of cases. Nuclear grooves were seen in 3 cases. Necrosis was extensive in 8 cases, focal in 10 cases, and was absent in 5 cases. In 8 cases, initial diagnoses included sex cord stromal tumor, germ cell tumor, sarcoma, or undifferentiated carcinoma. S-100 was positive in 18 of 19 cases, HMB-45 in 17 of 20 cases, MART-1 in 13 of 15 cases, tyrosinase in 10 of 15 cases, and Mitf in 8 of 14 cases. Inhibin was positive in 3 of 14 cases. Calretinin was focally positive in 1 of 12 cases. Treatment performed in 18 of the cases are as follows: oophorectomy with/without chemotherapy (10); total abdominal hysterectomy with bilateral salpingo-oophorectomy with/without chemotherapy (6); vaginal hysterectomy, bilateral salpingo-oophorectomy, and chemotherapy (1); and total abdominal hysterectomy with salpingo-oophorectomy (1). Follow-up ranging from 2 to 96 months was available in 18 patients. All but one had metastases in other organs, most often in the lungs. Thirteen patients died of disease (range 2-76 months), 3 are alive with disease (6-18 months), and 2 have no evidence of disease at 24 and 96 months; one was the patient with melanoma arising within a teratoma. In conclusion, MM involving the ovary is a rare disease, predominantly seen in women of reproductive age, and is associated with a poor prognosis. The tumor is most often metastatic from another site and is unilateral in most cases. Nodular or diffuse pattern and epithelioid cell type are most frequently seen, and the tumor can be mistaken for germ cell and sex cord stromal tumors. S-100 is the most sensitive marker. MART-1 was positive in the few cases that were negative with HMB-45. Inhibin can be focally positive in some cases.
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PMID:Malignant melanoma involving the ovary: a clinicopathologic and immunohistochemical study of 23 cases. 1516 69

We report a case of esophageal carcinoma that showed extraosseous accumulation of 99mTc-MDP in lymph node metastases to the cervical and paracardial lymph nodes. There are few cases showing abnormal extraosseous accumulation of 99mTc-MDP in esophageal cancer lesion. The patient was a 53-year-old man with advanced esophageal cancer. Bone scintigraphy demonstrated extraosseous accumulations in left supraclavicular and paracardial lymph node metastases. The histopathological diagnosis was small cell carcinoma of the esophagus, which is a rare disease with aggressive behavior and poor prognosis. Our patient underwent 2 courses of systemic chemotherapy (CDDP + VP16), but died of rapidly growing systemic metastases 5 months after the initial treatment.
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PMID:Extraosseous accumulation of 99mTc-MDP in lymph node metastases of small cell carcinoma of the esophagus. 1519 64

Thyroid carcinoma is a rare disease in children, and is mostly of the papillary histological type. It is often extended at presentation with frequent lymph node metastases. Treatment includes surgery (total thyroidectomy and lymph node dissection) and radioiodine therapy in case of extensive disease. Life long thyroxine treatment is given to all patients and when carefully controlled is devoided of adverse effects. Long term prognosis is favorable, but a few deaths have been reported some decades after initial treatment. Adverse prognostic indicators are younger age at discovery and presence of distant metastases.
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PMID:Follicular-cell derived thyroid cancer in children. 1525 Nov 53

Malignant melanoma of the vulva in childhood is a rare neoplasm. Lichen sclerosus of the vulva in childhood is also a rare disease. The association of these two rare lesions in the vulva of young girls is extremely rare. We present a 10-year-old white girl with malignant melanoma associated with lichen sclerosus of the vulva. She had dark pigmentation of both the labia minora and posterior fourchette. The inner labia majora and fourchette showed whitish, glistening areas of skin. Histologic examination found mostly an in situ lentiginous/mucosal melanoma with focal invasion to a depth of 0.44 mm in the left upper labium majus. All specimens showed evidence of lichen sclerosus. Partial vulvectomy was performed, and no metastases were detected at the time of treatment. The patient has been disease free for the 12 months after treatment. It is critical for physicians to realize that melanoma can occur in children, and although rare, can occur in the vulva. We feel that lichen sclerosus in this instance may represent a pattern of host immune response to melanoma.
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PMID:Malignant melanoma associated with lichen sclerosus in the vulva of a 10-year-old. 1591 84


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