Isolated (localized) idiopathic granulomatous (giant cell) vasculitis in an intramuscular lipoma.

Isolated (localized) idiopathic granulomatous vasculitis (IGV) is an uncommon, heterogeneous, and poorly defined group of disorders characterized by infiltration of the arterial wall caused by compactly grouped mononuclear phagocytes, with or without giant cells, in segmental distribution. We report on a 55-year-old woman with IGV limited to an intramuscular lipoma of the left thigh. The vasculitis was identified incidentally upon microscopic examination of the removed tumor. The IGV was centered on two medium-sized arteries, accompanied by narrowing of the lumens, and not associated with secondary changes such as infart or postinfart fibrosis. The inflammatory infiltrate was rich in T-lymphocytes and macrophages, with the presence of giant cells. The patient was asymptomatic and well in a follow-up period of 2 months, during which she was not treated. To our knowledge, this is the first report of lipoma involvement in localized IGV. It is important to distinguish cases of isolated intratumorous IGV from systemic disease, because the latter implies a poor prognosis and requires an aggressive treatment.

[Hormone expression and opioid receptors in fetal and adult lung]. / Expresión hormonal y de receptores opioides en pulmón fetal y del adulto.

OBJECTIVES: To describe the cellular distribution and level of expression of certain hormones and opioid receptors during fetal development and in the lung of the healthy adult. METHOD: We sampled lung tissue from fetuses at three stages of development (pseudoglandular, canalicular and saccular) (3 samples per stage), from newborn infants (3), from 10-month-old infants (2) and from adults (3) who had died without lung disease. After specific immunohistochemical staining for hormones (calcitonin, parathormone, serotonin and adrenocorticotropic hormone - ACTH) and opioid receptors, we assessed the percentage of positive cells for each cell type in each sample. RESULTS: Serotonin is the first to appear (pseudoglandular stage in isolated neuroendocrine cells) and it disappears later. Calcitonin appears in the canalicular stage in neuroendocrine and lung cells. Expression is at its peak at birth and is less in the adult lung. We found no ACTH or parathormone production. Opioid receptors appear in the canalicular stage and peak at birth. In adult lung, bronchiolar muscle and mesothelial cells, only delta-type opioid receptors are present. CONCLUSIONS: Pulmonary hormone secretion is significant during fetal development and peaks at birth. Calcitonin is the main hormone produced in the fetal lung. Opioid receptors are present during fetal development in various types of cells and peak at birth. An understanding of the expression of active substances could have therapeutic relevance in certain conditions, such as bronchial asthma or respiratory distress syndrome in the child.

Expresión hormonal y de receptores opioides en pulmón fetal y del adulto / Hormone expression and opioid receptors in fetal and adult lung

OBJETIVOS: Describir la distribución celular y el grado de expresión de diversas hormonas y receptores de opioides en el desarrollo embrionario y en el pulmón sano del adulto. MÉTODO: Seleccionamos tejido pulmonar de las tres etapas del desarrollo fetal (seudoglandular, canalicular y sacular, tres muestras por etapa), de recién nacidos (tres), niños de 10 meses (dos) y adultos (tres) fallecidos sin afección pulmonar. Practicamos tinción inmunohistoquímica para hormonas específicas (calcitonina, parathormona, serotonina y hormona adrenocorticotropa [ACTH]) y receptores de opioides tipo delta y mu. Valoramos el porcentaje de células positivas así como el tipo celular reactivo en cada caso. RESULTADOS: La serotonina es la primera en aparecer (estadio seudoglandular en células neuroendocrinas aisladas) para posteriormente desaparecer. La calcitonina aparece en el estadio canalicular en células neuroendocrinas y neumocitos. Su expresión máxima es al nacimiento y disminuye en el pulmón adulto. No hemos encontrado producción de ACTH ni de parathormona. Los receptores de opioides aparecen en la fase canalicular y alcanzan el máximo grado en el nacimiento. En el adulto sólo existen receptores para opioides tipo delta en neumocitos, células musculares bronquiolares y mesoteliales. CONCLUSIONES: La hormonosecreción pulmonar es importante durante el desarrollo fetal y alcanza su máxima expresión en el nacimiento. La principal hormona que produce el pulmón fetal es la calcitonina. Existen receptores opioides durante el desarrollo fetal en diferentes tipos celulares y alcanzan su máxima expresión al nacimiento. El conocimiento de la expresión de sustancias activas podría tener consecuencias terapéuticas en determinados procesos patológicos como el síndrome de distrés respiratorio en el niño o el asma bronquial (AU)

Concurrent tubulovillous adenoma and transitional cell carcinoma associated with diffuse gastric and intestinal metaplasia of the defunctioned ureter.

Villous adenoma is a common lesion of the gastrointestinal tract, but it is rare in the ureter. Thus, as far as we know, only one case limited to this location has been described. Intestinal metaplasia of the urothelium is not rare. However, only one case of gastric metaplasia with pseudopyloric glands has been described in the literature. We here report in detail on a tubulovillous adenoma of the ureter associated with diffuse gastric and intestinal metaplasia and a concurrent primary, solid, high grade transitional cell carcinoma, with extensive clear cell change, in a 56-year-old male patient. He had undergone a left nephrectomy for renal tuberculosis twenty years earlier, and the lesions developed in the ureteric stump. To the best of our knowledge, such a combination of lesions has not been reported previously either in the ureter or in the rest of the urinary tract. The coexistence of diverse lesions in our case might represent the pluripotentiality of the urothelium in association with chronic inflammation and neoplastic induction. The present report also emphasizes the metaplastic and malignant potential of a defunctioned urothelial structure. This case is of particular interest, because these coexistent lesions arose simultaneously with an anatomically separate adenocarcinoma of the rectum (Dukes' B). The patient died 76 days after admission. The dismal prognosis of our case was determined by the advanced anatomical stage and the histological high grade of the transitional cell carcinoma of the ureter.

Tumor de células de Leydig. Aportación de dos casos y revisión de la literatura / Leyding cell tumour. Two new cases, and review of the literature

El tumor de células de Leydig es el más frecuente de los tumores testiculares no germinales. La clínica de este tipo de tumores depende de la edad de presentación. Aportamos dos nuevos casos de Tumor de Células de Leydig, diagnosticados en un niño de 8 años con pseudopubertad precoz y en un adulto de 42 con ginecomastia. Realizamos una revisión de la literatura existente con respecto a esta rara patología y consideramos que la iconografía aportada es de gran interés para el conocimiento de la misma (AU)

Spindle cell rhabdomyosarcoma in adults. A case report and literature review.

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.

Symptomatic intracavitary (noninvasive) cardiac metastasis from low grade endometrial stromal sarcoma of the uterus.

We report a 49-year-old woman who was operated upon 33 years ago for uterine endometrial stromal sarcoma of low-grade malignancy. The patient showed obstruction of the right ventricular outflow tract. An echocardiogram and MRI showed that a mass emanating from the inferior vena cava filled the right atrium, the right ventricle, and part of the pulmonary artery. The entire intracavitary neoplasm was successfully removed and diagnosed as low-grade endometrial stromal sarcoma. To our knowledge, this is the second report of a low-grade endometrial stromal sarcoma reaching the heart via the inferior vena cava. In this case an intracavitary metastasis may be a feature indicating the progression of the endometrial stromal sarcoma. Therefore, this neoplasm should be included in the differential diagnosis of cardiac intracavitary neoplasms. Patients affected by this tumor may benefit from early identification and resection of the obstructing neoplasm.

Primary amyloid tumor (amyloidoma) of the jejunum with spheroid type of amyloid.

The present report documents an incidentally discovered, solitary amyloidoma of the jejunum in a 70 year old man with no predisposing disorder or other sites of deposition of amyloid, as was demonstrated at autopsy. Amyloid tumors of the intestine have been reported only rarely. In those reports the deposits were conventional in type, forming large masses of acellular, eosinophilic, homogeneous material. In the case described here the deposits were in the form of corpora amylacea-like structures, or spheroids. To the best of our knowledge, the corpora amylacea-like structures, or spheroid amyloid, have not been previously described in bowel amyloidoma. This rare form of amyloid deposition should be recognized so as to prevent misdiagnosis.

Amyloidosis of the rectum mimicking collagenous colitis.

Gastrointestinal involvement in cases of systemic amyloidosis is very common. In the colorectal mucosa, amyloid deposition is ordinarily seen around vessels or diffusely in the lamina propria. We report two cases in men aged 69 and 29 years, whose rectal biopsies revealed prominent subepithelial amyloid deposits mimicking collagenous colitis. The amyloid deposits were composed of AA protein. A review of the literature has yielded only one previously reported case. Pathologists should be aware of this deposition pattern of amyloid to prevent misdiagnosis. Our cases underline the importance of the routine use of histochemical stains for amyloid in all cases of colorectal biopsies showing histologic changes suggestive of collagenous colitis.