In adrenal insufficiency, recognition of the disease in its earlier phases may present a real challenge. The disease, if unrecognized and untreated, carries an almost uniformly poor and frequently fatal prognosis. We report a rare case of congenital adrenal aplasia accompanied by hyperpigmentation on the whole body surface. The diagnosis was confirmed by ACTH stimulation test and autopsy findings.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Autopsy ; Diagnosis ; Hyperpigmentation* ; Prognosis

A 5- year old female patient visited our clinic due to linear erythernatous and verrucous patches on the left extremities of 3 years duration, She was born with a small erythematous macule on the lateral side of left little finger. This macule, over 2 years, developed into pruritic, linear, and verrucous patches stretching from left little finger to lefr, axillai y area, and also erythematous, linear patches on the left thigh. On follow-up visit on October 1986, she has generalized psoriatic eruption with aggravation of originnl skin lesions. Digriosis was based on the clinical features and histopathnlogic findings of the skin lesions.
Extremities ; Female ; Fingers ; Follow-Up Studies ; Humans ; Nevus, Sebaceous of Jadassohn* ; Skin ; Thigh

The origin of Paget cells in extramammary Pagets disease is uncertain, however, some ultrastructural surveys revealed differentistion of Paget cells toward sweat gland apparatus. This is supported by recent immunohistochemical studies that Paget cells are irnmunohistochemically shown to react with antikeratin monoclonal antibodies specific to simple epithelia. In this siudy, seven cases of extramammary Pagets disease were immunohistochemically investigated with adenokeratin, carcinoembryonic antigen(CEA), involucrin by peroxidase-antiperoxidase(PAP) techniques. The results were as follow : l. Epidermis, hair follicies and sebaceous glands revealed negative immunoreacativity for adenokeratin in all cases. Sweat glands(6/7), Paget cells(6/7) and underlying adenocarcinoma revealed positive immunoreactivity for adenokeratin. 2. Epidermis, hair follicles and sebaceous glands(6/7) revealed negative immunoreactivity for CEA. Sweat glands, Paget cells and underlying adenocarcinoma revealed positive immunoreactivity for CEA in all cases. 3. Lower epidermis, sweat glands(6/7), Paget cells and underlying adenocarcinorna revealed negative irnmunoreactivity for involucrin. Upper epidermis, hair follicles and sebaceous glands(5/7) revealed positive immunoreactivity far involucrin. Based on these results, Paget cells of extramammary Pagets disease are considered to be derived from the sweat glands.
Adenocarcinoma ; Antibodies, Monoclonal ; Carcinoembryonic Antigen* ; Epidermis ; Hair ; Hair Follicle ; Paget Disease, Extramammary* ; Sebaceous Glands ; Sweat ; Sweat Glands

We report two cases of ectopic epididymal ducts and efferent ductules in the testicular appendices (TAs) of adult men with normally descended testes. In both cases, a sessile TA was incidentally found at the upper pole of the right testis during the scrotal hydrocelectomy. Microscopically, a few closely arranged tubules were detected within the TA. In the first case, the tubules were lined with a pseudostratified columnar epithelium with numerous, long microvilli, and were surrounded by a smooth muscle coat. In contrast, in the second case, the tubules had a wavy luminal surface, because ciliated columnar cells alternated with groups of cuboidal cells. In both cases, strong CD10 immunoreactivity was observed in the luminal border of the lining epithelium. Surgical pathologists should be aware of the presence of both ectopic epididymal ducts and efferent ductules that can occur in TAs, in order to avoid misinterpretation as transected, functional reproductive structures.
Adult ; Choristoma ; Epididymis ; Epithelium ; Female ; Humans ; Male ; Microvilli ; Muscle, Smooth ; Parovarian Cyst ; Phenobarbital ; Testis ; Wolffian Ducts

We have experienced three cases of extrarnsmmary Pagets disease. The first case was a 77-year-old female who showed 8x10cm sized, erythematous plaque on the suprspubic area of one year's duration. The second case was a 60-year-old male who showed erythematous, oozing, crusted, well circumscribed patches on the right scrotum and the pubic area of 8 years' duration. Third case was a 79-year-old male who showed erythernstous to gray, eczemstoid plaques with marginal elevation on the right side of the scrotum, penile root and pubic area of 3 years duration. On histologic exarnination, the third case showed swest gland adenocarcinoma in the dermis.
Adenocarcinoma ; Aged ; Dermis ; Female ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary* ; Scrotum

A case of linear IgA bullous dermatosis reported. A 32-year-old male with pruritic, papulovesicular eruptions on the whole body of 2 months' duration was examined. He had no history of diarrhea or fou]smellirig stool even though his diet cnntained gluten. A skin biopsy revealed subepidennal vesicles and papillary microabscesses infiltrated by many neutrophils and some eosinophils. Direct irnmunofluorescent examination of the perilesional skin showed a linear deposition of IgA along the basement mernbrane zone. The lesions responded rapidly to oral dapsone and topical steroid therapy but recurred whenever the oral mcdication was discontinued.
Adult ; Biopsy ; Dapsone ; Diarrhea ; Diet ; Eosinophils ; Glutens ; Humans ; Immunoglobulin A ; Linear IgA Bullous Dermatosis* ; Male ; Neutrophils ; Skin

BACKGROUND: The proto-oncogene c-met encodes the tyrosine kinase receptor for hepatocyte growth factor (HGF), which is a pleiotropic cytokine that controls growth, survival, motility, invasive migration, and differentiation of epithelial cells. Like several other epithelial neoplasms, thyroid carcinomas have been found to overexpress the c-met oncogene. We presently examine the expression of c-met protein in thyroid tumors and the correlation of c-met protein expression with prognostic factors in thyroid cancers. METHOD: We have examined the expression of the c-met oncogene in 62 paraffin-embedded thyroid cancer specimens (54 papillary carcinomas, 5 follicular carcinomas, 2 medullary carcinomas, and 1 anaplastic carcinoma), 20 benign tumors and 20 normal tissues using immunohistochemistry. We measured both the proportion and the intensity of stained cells and then calculated the staining index by multiplying the proportion and intensity scores. The staining index were categorized to be negative/low (staining index < or = 5) or high(staining index >5). The most important prognostic factors were age (over 45), tumor size (over 1.5 cm), lymph node metastasis, capsular invasion, vascular invasion and peripheral metastasis. RESULT: 1) The rate of expression of the c-met oncogene were 100%, 100% and 60% in thyroid cancer, benign tumors and normal thyroid tissue respectively. The expression of the c-met oncogene was restricted to the membrane. 2) The staining index of normal tissue, benign tumors and thyroid carcinomas was 1.8, 4.3 and 5.8 respectively. In malignancies, the staining index of papillary carcinoma was 5.7, follicular carcinoma 5.4, medullary carcinoma 7.5, and anaplastic cancer 9. 3) A high expression of c-met was not correlated with prognostic factors in papillary, follicular carcinomas or medullay carcinomas. CONCLUSION: The c-met oncogene might not play a role in the pathogenesis of thyroid neoplasia. There was no correlation between the high expression rate of the c-met oncogene and prognostic factors in papillary and follicular carcinomas.
Carcinoma, Medullary ; Carcinoma, Papillary ; Epithelial Cells ; Hepatocyte Growth Factor ; Immunohistochemistry ; Lymph Nodes ; Membranes ; Neoplasm Metastasis ; Neoplasms, Glandular and Epithelial ; Oncogenes* ; Protein-Tyrosine Kinases ; Proto-Oncogenes ; Thyroid Gland* ; Thyroid Neoplasms

BACKGROUND: Many oncogenes have been recently identified in human thyroid carcinomas, but the molecular mechanisms that lead to thyroid neoplasia are not well understood. To assess whether oncogene- encoded proteins can be regarded as useful prognostic indicators, we have evaluated the expressions of c-met, c-Ha-ras, c-myc oncogenes in patients with papillary thyroid cancer (PTC) in relation to the prog nostic factors. METHODS: We used immunohistochemistry to examine the expressions of c-met, c-Ha-ras, and c-myc oncogenes in 54 paraffin-embedded PTC specimens. We measured both the proportion (scale of 0-3) and the intensity (scale of 0-3) of the stained cells and then calculated the staining index (scale of 0-9) by multiplying the proportion and the intensity scores. The staining index was thus categorized as negative/low (staining index < or =5) or high (staining index >5). The considered prognostic factors were age (over 45), tumor size (over 1.5 cm), lymph node metastasis, capsular invasion, vascular invasion, and distant metastasis. RESULTS: 1) The rates of expression of c-met, c-Ha-ras, c-myc oncogenes were 100%, 81.5%, and 70.3% in papillary thyroid cancer and 100%, 30%, and 10% in benign tumors and 60%, 10%, and 0% in normal thyroid tissue, respectively. The expression of c-met oncogene was restricted to the membrane the expression of c-Ha-ras was stromal in 95.5% of the specimens, and that of c-myc was stromal in 94.7%. 2) High expression (staining index >5) of c-met, c-Ha-ras and c-myc were not associated with the prognostic factors such as age, tumor size, lymph node metastasis, capsular invasion, vascular invasion and distant metastasis. CONCLUSION: Although the rates of expression of c-met, c-Ha-ras, and c-myc oncogenes were high in papillary carcinomas (100%, 81.5%, and 70.3%, respectively), there was no relationship between the high expression rates of the oncogenes and prognostic factors.
Carcinoma, Papillary ; Humans ; Immunohistochemistry ; Lymph Nodes ; Membranes ; Neoplasm Metastasis ; Oncogenes* ; Thyroid Gland* ; Thyroid Neoplasms

We report the first case of an angiomyomatous hamartoma (AH) of the popliteal lymph nodes (LNs) occurring in association with diffuse pigmented villonodular synovitis (PVNS) of the knee. AH is a rare benign vascular disease with a predisposition for the LNs of the inguinal region. Twenty-five cases of AH have been reported to date; however, the precise pathogenesis is still undetermined. In the present case, an open synovectomy revealed two of three popliteal LNs in close proximity to the extra-articular component of diffuse PVNS. These LNs demonstrated irregularly distributed thick-walled blood vessels in the hilum. These vessels extended into the medulla and cortex and were associated with haphazardly arranged smooth muscle cells in the sclerotic stroma. These findings are compatible with an AH. Our observations raise the possibility that AH of the popliteal LNs may represent an abnormal proliferative reaction against the inflammatory process caused by PVNS of the knee.
Angiomyoma ; Blood Vessels ; Hamartoma ; Knee ; Lymph Nodes ; Myocytes, Smooth Muscle ; Synovitis, Pigmented Villonodular ; Vascular Diseases

No abstract available.
Bone Density* ; Gonads*