Timo intratiroideo ectópico como causa de nódulo tiroideo solitario / Ectopic intrathyroidal thymus as cause of a solitary thyroid nodule

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Adenomas sebáceos múltiples y carcinoma sebáceo estraocular: descripción de un caso / Multiple sebaceous adenomas and sebaceous carcinoma to estraocular: description of a case

El carcinoma sebáceo es un tumor maligno infrecuente derivado del epitelio de las glándulas sebáceas. Potencialmente puede desarrollarse de cualquier glándula sebácea, pero más comúnmente lo hace en el área periobitaria y de la cabeza y el cuello. Los carcinomas sebáceos extraoculares son tumores extremadamente raros, agresivos, malignos, con menos de 150 casos descritos.

Sideroblastic anaemia with reactive thrombocytosis versus myelodysplastic/myeloproliferative disease.

The reported incidence of thrombocytosis among cases of refractory anaemia with ring sideroblasts (RARS) may be as high as 15%. We report four additional cases of this association, which appear to be hematologically heterogeneous. One patient clearly represents a case of RARS with reactive thrombocytosis. Two cases have features suggestive of the coincidental occurrence of essential thrombocythemia and RARS. The fourth case could be best classified as a subtype of myelodysplastic/myeloproliferative disease unclassifiable (MDS/MPD U). Only new biological or molecular markers will allow better differentiation between these disorders.

[Cutaneous erythematous rash as first manifestation of T-cell prolymphocytic lymphatic leukemia]. / Erupción cutánea eritematosa como primera manifestación de leucemia linfática prolinfocítica de células T.

We present a case of a man 84 years-old, whose presentation feature was a cutaneous inespecific rash, and was diagnosed of T prolymphocytic leukaemia (T-PLL). In this review we analyze actual aspects concerning biology, diagnosis, classification, prognosis and treatment of this rare mature T cell leukaemia.

Erupción cutánea eritematosa como primera manifestación de leucemia linfática prolinfocítica de células T / Cutaneous involvement as the first manifestation of T-cell prolymphocytic leukaemia

Se presenta el caso de un varón de 84 años que debutó con una erupción eritematosa y en el cual se efectuó el diagnóstico de leucemia prolinfocítica T. Con este motivo, se revisan diversos aspectos actuales concernientes a la biología, diagnóstico, clasificación pronóstico y tratamiento de las leucemias de células T maduras. (AU)

Ventajas y deficiencias de la clasificación actual de los linfomas / Advantages and deficiencies of the current classification of lymphomas

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[Splenectomy indications at a general hospital]. / Indicaciones de esplenectomía en un hospital general.

BACKGROUND: To analyse the evolution of indications for splenectomy for a 11-year period. METHODS: A review was made of cases of splenectomy from clinical records and the records of the Pathology Service at Hospital General Universitario Gregorio Marañón from 1986 to 1996. Both adult and pediatric cases were included. RESULTS: From 1986 to 1996 there was a total of 354 patients (273 males and 162 females) who underwent splenectomy. Mean age of patients was 44.6 years (SD +/- 20.5 years) and there was an average of 39.5 cases per year. The most common causes were trauma (32%), "incidental" splenectomy (14%), thrombopenia (12%) and non Hodgkin lymphoma (11%). Compared with the other causes, the mean age of patients with trauma was lower than that of the other groups (p < 0.0001), in contrast with patients with non Hodgkin lymphoma or extra-splenic tumors, where it was higher (p < 0.0001 for both groups). A decline was observed of cases due to trauma from the first to the last year of the study (p = 0.0009), as well as an increase in the group of splenectomy related to extrasplenic tumors (p = 0.01). In the group of the HIV infected patients (n = 30), thrombopenia was the most common indication for splenectomy (11 cases). CONCLUSIONS: Although the average of splenectomy cases remains relatively constant, a decrease in cases due to trauma was observed and an increase of splenectomies on account of abdominal oncologic resections.

[The determination of monoclonality in B-cell lymphomas using the polymerase chain reaction technic on paraffin-embedded material]. / Determinación de monoclonalidad en linfomas B utilizando la técnica de reacción en cadena de la polimerasa semiacotada sobre material de parafina.

BACKGROUND: Clonal rearrangements of immunoglobulin are useful in the diagnosis of B-cell lymphomas. The value of the polymerase chain reaction (PCR) with consesuated primers in the variable (V) and joining (J) region is now well established. METHODS: Forty cases of B-cell lymphomas and 10 patients with reactive lymphoid proliferations have been studied. All cases were studied with two different semiacotated techniques, with similar primers in the J region (LJH and VLJH) and changing the primers of the V region (Fr2A and Fr3A). RESULTS: A 100% specificity and positive predictive value were obtained with both techniques. The sensitivity was 90% with Fr2A and 82% with Fr3A, but in 20% of the studies with Fr2A the extracted DNA was insufficient to perform the study while it could be done with Fr3A in all cases. The negative predictive value was 76% with Fr2A and 58% with Fr3A. CONCLUSIONS: Both techniques showed a good sensitivity, optimal specificity and positive predictive value, with low negative predictive value. Although the results are somewhat higher with Fr2A, this technique needs well preserved DNA and it can be an important set back when we deal with formaldehyde fixed, paraffin embedded tissues.

[Massive rectal hemorrhage caused by nonspecific colonic ulcers]. / Rectorragia masiva por úlceras cólicas inespecíficas.

Nonspecific colon ulcers are a rare disease which may produce lower gastrointestinal bleeding. A history of previous nonsteroidal anti-inflammatory drugs ingestion may be recorded in most patients. The right colon is the main location, being the cecum the most frequent site. However, they may be distributed in all the large intestine, including the rectum. We present a case of lower massive gastrointestinal hemorrhage produced by nonspecific colic ulcers requiring emergency total colectomy. Although infrequent, they have to be considered in every patient with lower gastrointestinal bleeding and previous nonsteroidal anti-inflammatory drugs ingestion.

Epstein-Barr virus detection in neck metastases by polymerase chain reaction.

Cervical nodal metastasis from occult carcinomas represents a diagnostic challenge. This is a common presentation of undifferentiated nasopharyngeal carcinoma (UNPC), but metastatic carcinomas from other sites must be considered. UNPC has the distinguishing feature of a close association with Epstein-Barr virus (EBV). Since the polymerase chain reaction (PCR) can detect EBV in archival tissues, it offers significant advantages over previous methods for the detection of viral genomes. Its extreme sensitivity allows analysis of small samples from needle aspirates. Using the polymerase chain reaction to amplify EBV sequences from archival tissues, 15 of 18 NPC samples were positive for EBV. Of these 18, 14 of 14 with UNPC were positive, 1 of 2 with squamous cell carcinoma (SCC) were positive, and 10 of 2 with adenocarcinoma were positive. All 6 UNPC metastatic to lymph nodes were positive. Carcinoma metastatic to cervical nodes from 17 of 17 non-UNPC occult primaries lacked EBV. This demonstrates the utility of EBV detection by the polymerase chain reaction in the evaluation of patients with metastases to neck nodes from occult primary carcinomas in order to identify cases of UNPC.

[Non-Mediterranean lymphoma producing heavy alpha chains]. / Linfoma no mediterráneo productor de cadenas pesadas alfa.

At the present, immunoproliferative small intestinal disease (IPSID) is considered a fairly homogeneous entity. Isolated heavy chain production is so closely related with IPSID that has been considered as a natural biologic marker for it. By contrast, we report here the case of a 63-year-old female that developed a multinodular small bowel lymphoma without clinical malabsorption symptoms. The main tumour mass was located in proximal jejunum and the neighbour intestinal mucosa did not show neoplastic cell infiltrates. Immunohistochemical methods demonstrated restrictive presence of IgA1 subclass in the neoplastic cells without presence of light chains. Cases as present are extremely infrequent and their potential relationship with IPSID has not been completely outlined, being useful in trying to understand the spectrum of gut lymphomas.

Sclerosing hemangioma of the lung. An immunohistochemical study of intermediate filaments and endothelial markers.

Three cases of so-called pulmonary sclerosing hemangioma have been studied for endothelial markers (alkaline phosphatase, adenosine triphosphatase, factor VIII-related antigen, and Ulex europaeus I lectin), for intermediate filaments (keratin, vimentin), and for carcinoembryonic and epithelial membrane antigen. Not one of the neoplasms expressed endothelial markers, carcinoembryonic antigen, or keratin reactivity. The tumor cells showed a positive reaction for epithelial membrane antigen and vimentin. The findings exclude an endothelial origin for this group of tumors and favored an epithelial origin as the probable genesis of the neoplastic proliferation.