1/5297. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease. ( info) |
2/5297. Multifocal choroidal malignant melanoma: at least 3 melanomas in one eye. OBJECTIVES: To describe a case of an enucleated eye harboring multiple choroidal malignant melanomas. methods: Clinical, ultrasonographic, and histopathological evaluations. RESULTS: Meticulous sectioning of the globe did not disclose any structural continuity between the 3 choroidal melanomas. Fourth and fifth masses were connected to one of the tumors by fibrous tissue and therefore were not considered as additional distinct lesions. CONCLUSION: Although very rare, a single eye can harbor multiple distinct choroidal melanomas. ( info) |
| We describe a patient in whom adenocarcinoma and lymphoma occurred simultaneously in the stomach. She presented with pain and lump in the epigastrium with history of hematemesis. endoscopy revealed a growth involving the lesser curvature, and biopsy showed poorly differentiated carcinoma. Histological examination of the gastrectomy specimen showed synchronous diffuse adenocarcinoma with primary lymphoma of MALT type. The latter entity is known to be associated with helicobacter pylori infection. ( info) |
4/5297. The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis. A 39-year-old Chinese man with hypertension being evaluated for elevated serum alkaline phosphatase (SAP) levels was found to have an incidental right adrenal mass. The radiological features were characteristic of a large adrenal myelolipoma. This mass was resected and the diagnosis confirmed pathologically. His blood pressure normalised after removal of the myelolipoma, suggesting that the frequently observed association between myelolipomas and hypertension may not be entirely coincidental. Persistent elevation of the SAP levels and the discovery of hypercalcaemia after surgery led to further investigations which confirmed primary hyperparathyroidism due to a parathyroid adenoma. The patient's serum biochemistry normalised after removal of the adenoma. The association of adrenal myelolipoma with primary hyperparathyroidism has been reported in the literature only once previously. Although unconfirmed by genetic studies this association may possibly represent an unusual variation of the multiple endocrine neoplasia type 1 syndrome. ( info) |
| We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor. ( info) |
6/5297. A case of long-term survival with stage IV small cell lung cancer and early-stage central-type squamous cell lung cancer treated by photodynamic therapy. The present report is on a 67-year-old man with stage IV small cell lung cancer and early-stage centrally located squamous cell cancer of the lung. He was diagnosed as small cell lung cancer with multiple metastasis to the ipsilateral lung and was found to have a central-type early-stage squamous cell cancer by bronchoscope. After obtaining a complete response to the small cell lung cancer with chemotherapy and radiotherapy, photodynamic therapy was applied to the squamous cell carcinoma, resulting in complete disappearance of the tumor. recurrence of small cell cancer occurred at the ipsilateral lung and this patient died of small cell cancer 8 years after initiation of treatment. Post mortem examination confirmed complete disappearance of squamous cell cancer treated by photodynamic therapy. This is a rare case of long-term survival with stage IV small cell lung cancer and early-stage central-type squamous cell lung cancer successfully treated by photodynamic therapy. ( info) |
7/5297. p53 and p16INK4A mutations during the progression of glomus tumor. Glomus tumors are significantly rare tumors of carotid body. The great majority of these tumors are benign in character. Here we present two brothers with hereditary glomus jugulare tumor who had consanguineous parents. radiotherapy was applied approximately 8 and 10 years ago for treatment in both cases. Eight years later, one of these cases came to our notice due to relapse. The mutation pattern of p53, p57KIP2, p16INK4A and p15NK4B genes which have roles in the cell cycle, was analyzed in tumor samples obtained from the two affected cases in the initial phase and from one of these cases at relapse. The dna sample obtained from the case in initial diagnosis phase revealed no p53, p57KIP2, p16INK4A or p15INK4B mutation. He is still in remission phase. Despite the lack of p53, p57KIP2, p16INK4A and p15INK4B mutation at initial diagnosis the tumor dna of the other case in relapse revealed p53 codon 243 (ATG-->ATC; met-->ile) and p16 codon 97 (GAC-->AAC; asp-->asn) missense point mutations. No loss of heterozygosity in p53 and p16INK4A was observed by microsatellite analysis of tumoral tissues in these cases. P53 and p16INK4A mutations observed in relapse phase were in conserved regions of both genes. No previous reports have been published with these mutations in glomus tumor during progression. The mutation observed in this case may due to radiotherapy. In spite of this possibility, the missense point mutations in conserved region of p53 and p16INK4A genes may indicate the role of p53 and p16INK4A in tumor progression of glomus tumors. ( info) |
8/5297. Primary endometrioid carcinoma of fallopian tube. Clinicomorphologic study. Twenty cases of primary Fallopian tube endometrioid carcinoma (PFTEC) are presented in the paper. This accounts for 42.5% of all histologic forms of primary Fallopian tube carcinoma (PFTC) found in our Department. The youngest patient was 38, and the oldest 68 years (mean: 56 years). Seven patients were nulliparas. Only two cases were bilateral. According to FIGO staging, 13 cases were evaluated as stage I, 4 as II, and 3 as stage III. Due to the histologic grading, 8 tumors were classified as well, 7 as moderately, and 5 as poorly differentiated. In the time of preparation of the manuscript, 12 women were still alive, 2 of them with recurrent disease. The follow-up of patients without recurrence ranged from 4 to 120 months (median: 63). Eight patients had died (survival time: from 4 to 65 months; median: 26). Metastases were found in 8 patients, especially to ovaries. In 14/20 cases of PFTEC various forms of tubal wall invasion were observed. Blood or lymphatic vessels involvement was found in 9 patients. Six of them had died and one is alive with the symptoms of disease. Immunohistochemical detection of the mutant form of p53 protein and oncogene product, c-erbB-2, was studied in 17 cases. Nine patients exhibited simultaneous p53 protein accumulation and c-erbB-2 expression. 2/9 of these patients are alive with recurrent tumors and 4/9 died. Endometrioid carcinoma of the Fallopian tube can be characterized by a tendency to superficial invasion of tubal wall and in a half of the cases by invasion of vessels. The majority of these tumors were diagnosed at an early stage tumors. ( info) |
9/5297. Synchronous and metachronous gastric adenocarcinoma: case report and literature review. Whilst synchronous adenocarcinoma of the stomach is well documented, metachronous primary disease is exceedingly rare. We report a man with a family history of colonic and gastric cancer, who underwent a resection of a Duke's C adenocarcinoma of the rectum, aged 56 years, and a proximal partial gastrectomy for synchronous stage 1 gastric adenocarcinomas of the lesser curve, aged 61 years. Nine years later, a metachronous gastric primary was discovered in the gastric remnant, necessitating total gastrectomy. Total gastrectomy is the operation of choice for synchronous gastric primaries as it ensures clearance and prevents metachronous growth. However, it may not be appropriate for all gastric cancer as operative morbidity and mortality are increased, and because synchronicity and metachronicity of gastric cancer are uncommon. Moreover, there are no consistent data to demonstrate a survival advantage for total compared with partial gastrectomy for operable gastric cancer. If, after partial gastrectomy, synchronous disease is detected in the resected specimen (as in this reported case), endoscopic surveillance for metachronous disease is advised, since this may be amenable to surgical cure. ( info) |
10/5297. Treatment of multiple glomangioma with tuneable dye laser. BACKGROUND: Multiple glomangiomas are a rare clinical occurrence. In the past, various treatments have been proposed for eradication of these tumours. OBJECTIVE: An alternative and effective therapy for multiple glomangioma is proposed. Results: The effective clinical response of multiple, painful, bluish-black lesions on the soles of the feet and the hands, diagnosed as multiple glomangioma, to flashlamp tuneable dye laser is reported. CONCLUSION: The flashlamp tuneable dye laser is an effective modality in the treatment of multiple glomangioma. ( info) |