[Retroperitoneal ganglioneuroma in childhood]. / Ganglioneuroma retroperitoneal en la infancia.

OBJECTIVE: An incidentally discovered retroperitoneal ganglioneuroma in a 4-year-old child is presented. METHODS: Ultrasonography and CT were performed. The surgical specimen was analyzed by macroscopic, histological and immunohistochemical techniques. RESULTS: US and CT localized a retroperitoneal mass independent from the left kidney and adrenal gland. The histological study showed a fascicular proliferation with myxoid and fibrillar areas mixed with mature ganglion cells. These cells were positive for neurofilament and neuron specific enolase. The patient had a favorable outcome with no signs of tumor recurrence. CONCLUSIONS: Ganglioneuroma is a rare, completely mature tumor that has to be differentiated from neuroblastoma.

[Intestinal tuberculosis, a difficult suspected diagnosis]. / Tuberculosis intestinal, un difícil diagnóstico de presunción.

Intestinal tuberculosis (ITB) is an always difficult clinical diagnosis; we must keep in mind it in the diagnosis of patients with abdominal pain, even without concomitant lung involvement. Seven patients with ITB were revised. Five patients were male and two female, with a mean age of 45 years; all of them presented abdominal pain. Ileocecal region was involved in 71.4% of the cases. Only one patient showed old specific pulmonary lesions radiologically. Neoplasia was the most frequent clinical presumption. ITB diagnosis was made only in one case, as first possibility. Histopathological examination revealed caseous granulomas in all the cases, with acid-alcohol resistant bacilli in 71.4% of them. We want to underline the need of thinking about ITB, in the clinical evaluation of in specific abdominal pain, above all in immunosuppressed patients, and to emphasize the importance of histopathologic findings in the final diagnosis of this entity.

[An aggressive inguinal (parafunicular) angiomyxoma in a male patient]. / Angiomixoma agresivo inguinal (parafunicular) en paciente varón.

OBJECTIVE: To report a case of aggressive inguinal angiomyxoma in a male patient. METHODS: An 82-year-old male patient presented with a well-defined, 6 cm. parafunicular mass in the right groin. The mass was located adjacent to the spermatic cord and had been noted 8 years earlier. Patient evaluation included CT, ultrasound and immunohistochemical studies. RESULTS: The CT and US findings suggested lymph node enlargement. Microscopic analysis showed a myxoid tumor with partially infiltrating margins, vascular channels of small-sized vessels with thick walls occasionally with hyalinization and spindle-shaped or stellate mesenchymal cells with ill-defined margins without atypia or mitosis that were positive for vimentin and negative for actin, desmin, keratins, CD34 and protein S-100. No tumor recurrence or metastasis has been observed at 26-months' follow-up. CONCLUSIONS: To our knowledge, this is one of the few cases of inguinal angiomyxoma in male patients; 16 have been reported to date. This neoplasm appears to originate from pelvic soft tissue fibroblasts.

Two new cases of Cumming syndrome confirming autosomal recessive inheritance.

We report on two stillborn sisters with generalized hydrops, campomelia, cervical lymphocele, and polycystic dysplasia of kidney, liver, and pancreas. This syndrome conforms to that first described by Cumming et al. [Am. J. Med. Genet. 25:783-790, 1986]. This observation provides additional support for the notion that this syndrome has an autosomal recessive pattern of inheritance.

[An intimal sarcoma of the pulmonary artery. An immunohistochemical study]. / Sarcoma intimal de la arteria pulmonar. Estudio inmunohistoquímico.

Pulmonary artery intimal sarcomas tend to be presented with symptoms of pulmonary thromboembolism and grow regionally, with little capacity to metastasize. They probably originate from subendothelial cells, that become myofibroblasts. Knowledge of it is important to establish a presurgery diagnosis, with the possibility of a total resection, the only useful treatment until now. We report a case of a pulmonary artery primary sarcoma, in a 73 year old woman, admitted with hemoptysis and pleuritic chest pain, who died ten days after. Autopsy revealed an intraluminal mass at the pulmonary artery trunk, without regional nor distance involvement. Microscopic study showed a pleomorphic tumor with spindle and epithelioid cells, positive for actin, desmin and vimentin. All these data support the diagnosis of primary intimal sarcoma of the pulmonary artery. We want to emphasize the myogenic differentiation of the tumor, uncommon in previously reported cases.

[Inflammatory pseudotumor of the spleen. An old concept with many questions]. / Pseudotumor inflamatorio de bazo. Un viejo concepto con múltiples interrogantes.

Inflammatory pseudotumor of spleen is an infrequent benign condition. It is difficult to differentiate, on a clinical and radiological basis, from haematologic neoplasms, granulomatous diseases as sarcoidosis and splenic hamartoma. Sometimes can be an incidental finding. Two women, aged 72 years, are presented. On the first case the sympthons mi micked a malignant disease. The second one was an incidental finding in a routine study for cholecystitis. Histological and immunohistochemical study showed a polymorphic cellular population including plasma cell, lymphoid cells, histiocytes, eosinophils and spindle cells, showing a reactive benign character. Plasma cells presented light chains polyclonality. Lymphoid cells were mature and with T inmunophenotype. Spindle cells were focally positive for muscle spe-cific actin and vimentine. In the first case, ultraestructural study showed myofibroblast morphology on the stromal spindle cells. Like many other authors have already postulated, immunohistochemical and ultraestructural findings would corroborate the mesenchymal reactive and benign nature of this type of lesions.

[Alveolar rhabdomyosarcoma with massive infiltration of the bone marrow as its initial manifestation]. / Rabdomiosarcoma alveolar con infiltración masiva de medula ósea como manifestación inicial.

We report a case of alveolar rhabdomyosarcoma (AR) with massive infiltration of bone marrow at presentation, and initial diagnosis in bone marrow aspirate. A 35 year old man presented with a submandibular mass, and hematomas after mild traumatisms. Peripheral blood showed thrombocytopenia and a normocytic anaemia. Bone marrow film showed diffuse involvement by undifferentiated blasts with rhabdomyoblastic features. Subsequent biopsy of submandibular lymph node confirmed the diagnosis with positivity for specific muscle actin and desmin, and negativity for lymphoid markers. Initial presentation of AR with extensive bone marrow involvement is extremely rare, and it could lead to wrong diagnosis and treatment of acute leukaemia, with the serious consequences that this would have. Immunohistochemical study and morphologic differential features can be of great diagnostic help.

[Extramedullary anaplastic plasmacytoma]. / Plasmocitoma anaplásico extramedular.

A case of extramedullary plasmacytoma of the maxillary sinus, locally aggressive, in a 65 years old man is presented. Clinical diagnosis of adenocarcinoma was suspected but the pathological study showed an anaplastic morphology, with little plasmacytic differentiation and lack of stain with cytokeratins and epithelial membrane antigen (EMA), immunohistochemical marker of plasmatic cells, among others. Definitive diagnosis was based on light chain restriction and lack of multiple myeloma.

Parotid metastasis from renal clear cell adenocarcinoma. An unusual site for metastasis.

Metastasis to the parotid region is rare, and originates primarily from head and neck squamous cell carcinoma and melanoma of the skin. Renal clear cell adenocarcinoma has considerable metastatic potential and the parotid gland is one possible destination. Histopathologic study is important to differentiate this entity from primary clear cell parotid neoplasm.

[Primary round-cell liposarcoma of the bladder]. / Liposarcoma de células redondas primario de vejiga urinaria.

OBJECTIVE: To report a rare case of primary round cell liposarcoma of the urinary bladder. METHODS: A case of primary round cell liposarcoma of the urinary bladder in an 80-year-old female with hematuria is presented. The patient had a large, solid, non-circumscribed bladder tumor of 6.5 cms. The radiological, histological and immunohistochemical findings are discussed. RESULTS: The neoplasm was composed of isolated lipoblasts among numerous small round cells that were positive for S-100 and vimentin, and a high proliferation rate was demonstrated by Ki-67. The patient died 10 months after the histologic diagnosis. CONCLUSION: Primary liposarcoma of the urinary bladder is a very rare tumor with a poor prognosis that usually presents as a large tumor mass.

p53 expression is of independent predictive value in lymph node-negative breast carcinoma.

The aim of this study was to evaluate p53 expression, determined by immunohistochemistry, in 151 infiltrating ductal breast carcinomas with negative axillary lymph nodes, and to determine whether p53 can be considered as an independent prognostic value for overall and disease-free survival. A monoclonal antibody (DO-7) that reacts with an epitope on the N terminal portion of the human protein p53 was used to detect p53 in paraffin-embedded sections, utilising a standard avidin-biotin-peroxidase complex (ABC) technique with a microwave oven antigen retrieval. Overexpression of p53 (more than 50% of stained cells) was found in 45 cases (30%). Forty-five cases were negative and occasionally or moderately stained cells were present in 61 cases. p53 protein overexpression was significantly associated with high histological grade and tumour necrosis, high MIB-1 value (MIB-1 > 30%) and negative oestrogen receptor status. Univariate analysis (log-rank) showed a shorter overall survival (P = 0.003) in patients with high tumour p53 positivity. This statistical significance was also seen on multivariate analysis (Cox's logistic regression, P = 0.004). p53 protein overexpression is an independent prognostic marker in node-negative breast carcinoma for overall survival and should be used with other prognostic factors.

[Cytological diagnosis of malignant melanoma of the esophagus]. / Diagnóstico citológico de melanoma maligno de esófago.

Malignant melanoma of the esophagus is an extremely rare neoplasm. We presented a case of this neoplasm diagnosed on a cytologic smear from a 84 year-old women. The smear was characterized by scattered dissociated cells with hyperchromatic irregular nuclei, prominent nucleoli and occasional cytoplasmic brown pigment. The immunohistochemical study revealed positivity for HMB-45 and S-100 protein.

[Bilateral isolated infiltration in the epididymis and sperm cord as first sign of chronic lymphatic leukemia]. / Infiltración aislada bilateral de epidídimo y cordón espermático como primer signo de leucemia linfática crónica.

OBJECTIVE: Involvement of extralymphatic tissue in chronic lymphocytic leukemia (CLL) is uncommon and that of the epididymis and spermatic cord is rare. This paper describes an unusual case with metastasis to both epididymis and the spermatic cord as the first sign of reactivation of CLL. METHODS/RESULTS: A patient with CLL is described. The first sign of disease progression was the involvement of both epididymis and the spermatic cord. The literature on tumors of the epididymis and spermatic cord and CLL is briefly reviewed. CONCLUSION: Metastatic tumors of the epididymis and spermatic cord are rare. The importance of the pathological findings in making the diagnosis is underscored.

[KI-1-positive, anaplastic, large-cell lymphoma related to Hodgkin's disease]. / Linfoma anaplásico de células grandes Ki-1 positivo relacionado con Hodgkin.

We report a case of lymphoma associated with lung carcinoma that shows morphological and immunohistochemical features of anaplastic large cell Ki-1 positive lymphoma and Hodgkin's disease, with positivity for Ki-1 (CD-30) (characteristic of both lymphomas) and Leu-M1 (CD-15) (normally dosent absent in anaplastic lymphoma). This subtype of lymphoma is designated anaplastic large-cell Hodgkin's related lymphoma (ALCL related to HD) and is considered by some authors as a secondary anaplastic large-cell lymphoma.

[Neuroendocrine carcinoma of the ampullar region with oat-cell histological features and adenocarcinoma]. / Carcinoma neuroendocrino de la región ampular con rasgos histológicos de "oat-cell" y adenocarcinoma.

We report a case of primary duodenal carcinoma showing neuroendocrine and glandular differentiation, in a 65-years-old male. Both components were closely related and transition between them was observed. The neuroendocrine component was composed of an undifferentiated "oat-cell" type, with positive immunostaining for neuroendocrine and epithelial markers. Ultrastructural findings confirmed this double differentiation, with dense cytoplasmatic granules of neurosecretory type. The neuroendocrine component showed greater aggressiveness with lymph node metastasis.

Diaphysitis in tumoral calcinosis syndrome.

Diaphysitis is uncommon in children. We describe a case of diaphyseal inflammation in several bones with high levels of phosphorus in an 8-year-old boy who later developed subcutaneous masses of tumoral calcinosis. This report serves as a review of this puzzling problem in clinical diagnosis.

[Coexistence of chronic lymphoid leukemia and Hodgkin's disease. Immunohistochemical study]. / Coincidencia de leucemia linfoide crónica y enfermedad de Hodgkin. Estudio inmunohistoquímico.

We report a new case of coincidence of chronic lymphocytic leukemia (CLL) and mixed cellularity Hodgkin's disease (HD) in the same lymph node. These findings were corroborated by immunohistochemical study showing Reed-Sternberg (RS) cells, with typical immunophenotype in an inflammatory background, closely related to a small neoplastic lymphoid cell population. This is an uncommon feature, and, to our knowledge, this is the first case published in the Spanish literature.