Linfoma cutáneo de células B de la zonamarginal y sarcoidosis: presentación simultánea / Cutaneous marginal zone B-cell lymphoma and sarcoidosis: simultaneous presentation

El síndrome sarcoidosis-linfoma es la asociación en un mismo paciente de sarcoidosis y un linfoma. Describimos un paciente de 32 años que acude por tres lesiones nodulares cuya biopsia es diagnóstica de linfoma cutáneo de células B de la zona marginal. Durante el estadiaje se detectaron adenopatías mediastínicas cuya biopsia mostró granulomas no necrotizantes compatibles con sarcoidosis. Este sería el primer caso descrito que asocia linfoma cutáneo B de la zona marginal con sarcoidosis de forma simultánea (AU)

The sarcoidosis-lymphoma syndrome is the coexistence of sarcoidosis and malignant lymphoproliferative disease in the same patient. We describe a 32-year-old man who presented with three cutaneous nodular lesions. Skin biopsies revealed marginal zone cutaneous B-cell lymphoma. Radiologic Studies showed hiliar lymphadenopathy that histologically revealed non-caseating granulomas consistent with sarcoidosis. This report is the first case of simultaneous ocurrente in the same patient of primary cutaneous marginal zone B cell lymphoma and sarcoidosis (AU)

Dermatofibrosarcoma protuberans congénito: descripción de un caso / Congenital dermatofibrosarcoma protuberans: a case report

El dermatofibrosarcoma protuberans es un tumor fibrohistiocitario de bajo grado poco común y que suele aparecer en adultos entre los 20 y 50 años de edad. Inicialmente se presenta como una mácula o placa violácea de aspecto vascular sobre la que aparecen posteriormente lesiones nodulares en la superficie. El diagnóstico es histológico y se trata de un tumor de células fusiformes que se disponen en fascículos cortos adoptando un patrón arremolinado característico. Es un tumor con una elevada tasa de recurrencias locales, aunque son raras las metástasis. El tratamiento es quirúrgico con amplios márgenes. Este tumor excepcionalmente afecta a niños menores de 16 años y todavía más raros son los casos congénitos, con sólo 27 casos descritos en la literatura. Se describe el caso de un niño de 10 años de edad con un dermatofibrosarcoma protuberans presente desde el nacimiento y sin signos de recurrencia en la actualidad

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence

[Congenital dermatofibrosarcoma protuberans: a case report]. / Dermatofibrosarcoma protuberans congénito: descripción de un caso.

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence.

Atrophic dermatofibroma accompanied by aneurysmatic characteristics.

A 40-year-old man presented a painful haemorrhagic plaque on his chest in the same location where a nodular lesion had been presented for many years. After 2 months, the plaque was replaced by a depressed lesion. The lesion diagnosed as an anetoderma was excised and the biopsy showed an atrophic dermatofibroma accompanied by aneurysmatic characteristics.

Subepithelial myofibroblasts and tenascin expression in microscopic colitis.

AIMS: To assess differences in the pattern of subepithelial myofibroblasts and the expression of tenascin as a marker of extracellular matrix production in collagenous and lymphocytic colitis. METHODS AND RESULTS: Colorectal biopsies were studied from 122 patients with chronic diarrhoea and normal colonoscopy. The pathological diagnoses were collagenous colitis (n = 35), lymphocytic colitis (n = 37), mild non-specific chronic inflammation (n = 28) and normal mucosa (n = 18). Four cases showed features of collagenous colitis but with collagen bands <10 micro m thick. Normal mucosa from 14 patients without diarrhoea served as healthy control tissue. Immunohistochemical expression of alpha-smooth muscle actin (myofibroblast marker) and tenascin was evaluated in well-orientated sections. The expression of alpha-smooth muscle actin was significantly increased in collagenous colitis compared with all the other groups. Strong tenascin subepithelial expression was seen in all cases of collagenous colitis, including the four without full-blown features. The mean thickness of tenascin bands was greater than that obtained by conventional stains. CONCLUSIONS: There are clear differences, with respect to extracellular matrix remodelling, between collagenous and lymphocytic colitis. These results support the theory of matrix overproduction in the genesis of collagenous colitis.

[Eosinophilic ulcer of the oral mucosa: 11 cases]. / Ulcère éosinophilique de la muqueuse buccale: 11 cas.

BACKGROUND: The eosinophilic ulcer is a lesion of the oral mucosa, that has been infrequently described in the literature. This is a benign and self-limiting lesion of unknown origin. CASE REPORTS: In this article, eleven new cases of eosinophilic ulcer of the oral mucosa are presented. The clinical, histologic and evolutive features are reviewed. DISCUSSION: Recurrent trauma is clearly involved in the pathogenesis of this entity.

Subcutaneous nodules in Whipple's disease.

BACKGROUND: Cutaneous findings other than hyperpigmentation are rare in Whipple's disease. CASE REPORT: We present the case of a 59-year-old man previously diagnosed with Whipple's disease by duodenal biopsy, who developed red-brown, painful, subcutaneous nodules on the buttocks, thighs, arms and legs. Biopsy of these nodules showed a septal panniculitis and foamy macrophages containing PAS-positive, diastase resistant intracytoplasmic material, characteristic of Whipple's disease and similar to that observed in the duodenal biopsy. Ultrastructurally, this material in the histiocytes corresponded to degenerated bacilli. CONCLUSIONS: This is the fourth documented case of subcutaneous involvement by Whipple's disease. One should consider the possibility of Whipple's disease in any patient who presents with symptoms compatible with that condition who demonstrates septal panniculitis with a large amount of foamy histiocytes.

[Gastrointestinal stromal tumors: prognostic value of DNA analysis with flow cytometry and of classical clinicopathologic parameters]. / Tumores mesenquimales del tubo digestivo: valor pronóstico del análisis del ADN por citometría de flujo y de los parámetros clinicopatológicos clásicos.

BACKGROUND: Biological behavior of gastrointestinal stromal tumours (GIST) is still difficult to predict. The aim of this study was to analyze the prognostic valve of DNA study by flow cytometry in addition to the classical clinicopathologic parameters. PATIENTS AND METHODS: 82 GIST were investigated to assess by multivariable analysis the prognostic significance of classical morphologic and clinical features and DNA flow-cytometric analysis. RESULTS: Features significantly correlated with bad prognosis were: pain, recurrences, metastasis, small intestine tumours, invasion of adjacent tissues, size > 6 cm, infiltrative pattern, high cellularity, high-grade of atypia, mitosis count > 2/50 high powered field, necrosis, DNA aneuploid-hyperploid or near-diploid, S and SG2M-phase of aneuploid/s population/s > 11% and average S-phase > 11%. Multivariable analysis showed that mitotic activity was the only independent variable indicating poor prognosis. CONCLUSIONS: In this series of GIST mitosis count was the only independent prognostic factor, being more important than flow-cytometric variables like DNA ploidy and cellular cycle values.

Localized fibrous tumor of the pleura: immunohistochemical and flow-cytometric DNA study.

This report describes three cases of localized fibrous tumor of the pleura (LFTP) with clinical and pathological differences. Case 1 presented with symptoms; the tumor size was 6.5 cm and it was attached to the lung and chest wall; histologically it was composed of spindle cells with pleomorphism, mitoses, hemorrhage and necrosis. Cases 2 and 3 were casual findings and consisted of well-circumscribed tumors of 3 and 8.5 cm, respectively; histologically both showed hypocellularity. All cases exhibited positive stains for vimentin and negative for keratin. These results, added to histological features, suggested a mesenchymal origin. Flow cytometry quantitation of DNA disclosed a diploid pattern in all three cases with a small "near diploid" cell population additionally in Case 1; the S-phase fraction was low in all cases. These findings, that could be considered favourable prognostic signs, and the complete tumoral resection performed in the three tumors, could explain the absence of recurrences after 32, 27 and 19 months, respectively.

Polyostotic coexisting fibrous dysplasia and aneurysmal bone cyst of the chest wall.

A case of a 39-year-old woman with a palpable mass in the right hemithorax is presented. The mass had been growing during the last 16 years. Radiographs and computed tomography showed two lesions in the right thoracic wall: the greater was in the anterior and lateral portion of the 7th rib, the minor lesion in the 6th rib costovertebral joint. Both lesions were surgically removed. Histological examination demonstrated the association of fibrous dysplasia and aneurysmal bone cyst in the two lesions. The coexistence of these two lesions supports the theory that aneurysmal bone cyst may represent a secondary change due to haemodynamic alterations of the vascular bed caused by fibrous dysplasia.

[Extrabronchial small-cell carcinoma: apropos 6 cases]. / Carcinoma de célula pequeña extrabronquial: a propósito de 6 casos.

Extrabronchial small cell carcinoma (ESCC) is an infrequent tumor with controversial histogenesis, clinical evolution and therapeutic strategy. The aim of this study was to know the immunohistochemical features and the clinical evolution of patients diagnosed of ESCC during a 10 year period. All the diagnoses of small cell carcinoma (bronchial and extrabronchial) carried out by the Unit of Pathology between 1980-1989 were reviewed. In all the ESCC an immunohistochemical study was performed with three neuroendocrine markers, chromogranin, neurospecific enolase and synaptophysin. The clinical evolution of the patients is described. The 6 patients with ESCC represented 4.7% of all the small cell carcinomas. The primary localization was: parotid, urinary bladder, the skin, maxillary sinus and esophagus (2 patients). In five cases positivity was observed for one or more of the neuroendocrine markers. In two cases the ESCC was associated with differentiated cell populations (squamous carcinoma). The diagnosis of ESCC logically obliges the bronchial origin and the presence of ectopic hormonal secretion syndromes to be discarded. The administration of chemotherapy regimes used in small cell lung carcinoma is advised.

Fine-needle aspiration biopsy of hepatic papillary cystadenocarcinoma in Caroli's disease.

A case of papillary adenocarcinoma arising in Caroli's disease (CD) in a 25-yr-old woman is reported. The diagnosis of malignancy was made by ultrasound-guided, fine-needle aspiration biopsy (FNAB). As there were no metastasis, a liver transplant was performed and the diagnosis was histologically confirmed. Only 22 cases of malignant transformation of CD have been reported to date, all among middle-aged subjects (mean age: 52 yr) of both sexes. Most of these reported cases (83%) were found to be bile duct adenocarcinomas with occasional reports of hepatocellular and undifferentiated carcinomas. Our case is the youngest reported to date and the first to be diagnosed cytologically.

[Hypoplasia and pulmonary hypertension in Down syndrome: prognostic evaluation using pulmonary biopsy]. / Hipoplasia e hipertensión pulmonar en un síndrome de Down: valoración pronóstica mediante la biopsia pulmonar.

The pulmonary hypoplasia, in Down's syndrome with congenital cardiac malformation, probably explains the poor behaviour of these patients, leading to early vascular pulmonary lesions, which progress quickly. We present a case, in which the lung biopsy, done before cardiac surgery, showed a lung hypoplasia and also enabled us to establish the grading of the pulmonary vascular disease. The pathologic evaluation is necessary to decide upon the intracardiac repair and to predict the child's outcome.