Management of non-hepatic distant metastases in neuroendocrine neoplasms.

Neuroendocrine neoplasms represent an uncommon disease with an increasing incidence. Thanks to improvements in diagnostic and therapeutic methods, metastases previously considered uncommon, such as bone metastases, or even very rare, such as brain, orbital and cardiac metastases, are more frequently found in daily practice. Due to the great heterogeneity of these neoplasms, there is a lack of high-quality evidence on the management of patients with these types of metastases. The aim of this review is to provide the current state of the art, reviewing neuroendocrine neoplasm specific studies and useful information from other tumor types and to propose a treatment recommendation with algorithms to consider in daily clinical practice.

Primary neuroblastoma presenting as a paratesticular tumor.

A 3-month-old boy was operated on for an inguinal tumor. Histological diagnosis was neuroblastoma. This is the second known case of primary paratesticular neuroblastoma reported in the literature. The treatment of choice for Evan's stage I is surgery.

[Analysis of proliferating cell nuclear antigen (PCNA) expression in 24 cases of primary non-small cell pulmonary carcinomas and correlation with survival]. / Análisis de la expresión del antígeno nuclear de proliferación celular (PCNA) en 24 carcinomas primarios de pulmón de células no pequeñas y correlación con la supervivencia.

Proliferating cell nuclear antigen (PCNA) is a 36 kD nuclear protein involved in DNA replication that is believed to provide an indication of proliferation in some neoplasms. This study analyzes PCNA expression in 24 cases of primary non-small cell lung cancer using monoclonal PC-10 antibodies in paraffin embedded material. We found significant inter- and intra-tumoral variations in PCNA expression, and no statistically significant relation between the amount of PCNA expression and the size and location of tumors, index of mitosis, histological tumor type or patient age. We found a statistically significant relation (r = 0.47; p < 0.05) between survival and amount of PCNA expression in a sample of 19 cases, but no statistically significant differences in survival related to whether PCNA expression was slight (0-25), moderate (25%-50%) or high (> 50%), and no prognostic value for degree of PCNA expression.

[Atypical presentation of syringocystoadenoma papilliferum]. / Siringocistoadenoma papilífero de presentación atípica.

Syringocystoadenoma papilliferum is benign adnexal tumor derived from the sweat glands that is located in the head and neck in 70-80% of patients, and commonly presents as a papule or a solitary nodule. We report a case of syringocystoadenoma papilliferum with an atypical presentation given its location in the thigh and the peculiar histologic features, unrepresentative of this entity.

Focal hyperplastic hepatocellular nodules in hepatic venous outflow obstruction: a clinicopathological study of four patients and 24 nodules.

AIMS: In hepatic venous outflow obstruction (Budd-Chiari syndrome), focal hepatocellular nodules are occasionally discovered showing variable morphology. These could be interpreted either as neoplastic (adenoma), regenerative (large regenerative nodule) or reactive to abnormal vasculature (focal nodular hyperplasia). The aim of this study was to investigate their histogenesis and to determine their morphological characteristics in order to provide diagnostic criteria. MATERIAL AND METHODS: Twenty-four hepatocellular nodules were studied, which were found in three explanted livers and in one additional autopsied liver from four patients with Budd-Chiari syndrome. As controls, we employed three explanted livers without nodules from patients who also suffered from Budd-Chiari syndrome. We attempted to classify the nodules morphologically as either adenoma-like, large regenerative nodule or focal nodular hyperplasia-like, using criteria from the literature. RESULTS: Out of the four cases, we observed two nodules in each of two livers, five in the third one and up to 15 in the remaining one. The size of the nodules ranged from 4 to 25 mm. Eleven nodules could be categorized as large regenerative nodules (two of them with a central scar), seven as focal nodular hyperplasia-like and six as adenoma-like. Some large regenerative nodules showed proliferated arteries with muscular hyperplasia similar to that seen in focal nodular hyperplasia. In the individual livers we could find nodules of various categories. Patchy or diffuse monoacinar regeneration was seen in most cases (six out of seven cases) in the macroscopically non-nodular liver parenchyma. In addition, thrombotic obstruction of portal vein branches was present in all except one of the nodular cases, but in none of the controls. Thus, it appears that portal venous obstructions are frequently, but not invariably associated with the development of nodules. CONCLUSIONS: The hepatocellular nodules seen in livers from patients with Budd-Chiari syndrome share morphological characteristics with large regenerative nodules, focal nodular hyperplasia and hepatocellular adenomas. Their multiplicity, the existence of mixed lesions, the frequent hepatocellular regenerative background as well as the frequently associated portal venous obstructions suggest that these nodules are regenerative in nature and conditioned by an uneven blood perfusion throughout the liver. In their differential diagnosis, the clinicopathological context in which they occur is of paramount importance and should allow recognition that those resembling adenomas may not be true neoplasms.

Collagenous gastrobulbitis and collagenous colitis. Case report and review of the literature.

A case is reported of collagenous gastrobulbitis on collagenous colitis in a 57-year-old woman with a 6-month history of watery diarrhea. Low serum levels of total proteins and albumin and increased fecal elimination of alpha1-antitrypsin were the only abnormal laboratory test results. Biopsy specimens from the colon, rectum, antrum, fundus, and duodenal bulb showed a thick subepithelial band composed of ultrastructurally normal collagen immunohistochemically negative for collagen IV and laminin. The diarrhea resolved with prednisone and responded to this treatment after a relapse 6 months later. One year later the patient developed severe alimentary intolerance and secondary weight loss. This symptom also responded to the same treatment. However, the collagen deposition did not disappear in the second biopsy samples of colonic and gastric mucosa. Only six cases have been previously reported with gastric and/or duodenal subepithelial collagenous deposition. Four were associated with collagenous colitis. One of these presented a subepithelial collagenous band in the terminal ileum. All these features suggest that this collagen deposition may affect the entire digestive tract with variable intensity, extension, and symptoms.

Dermatofibroma parasitized by Leishmania in HIV infection: a new morphologic expression of dermal Kala Azar in an immunodepressed patient.

Visceral leishmaniasis is a protozoan infection that may complicate the course of patients with human immunodeficiency virus (HIV). Dermatofibroma is a cutaneous fibrohistiocytic lesion considered neoplastic by some authors and inflammatory by others. Eruptive dermatofibromas have been described in patients with HIV infection or with other altered immunity situations. We present the case of a 32-year-old, HIV-positive man with visceral leishmaniasis who complained of the appearance of a cutaneous lesion in the leg formed by the coexistence of dermatofibroma and Leishmania parasitic colonization. As far as we know, this type of association has not been reported previously. We consider that the dermatofibroma could have developed as an unusual form of fibrohistiocytic reaction to leishmania. From a practical approach, we recommend the search of leishmaniasis in dermatofibroma in immunosuppressed patients.

Hilar biliary cysts in hepatic transplantation. Report of three symptomatic cases and occurrence in resected liver grafts.

Hilar cysts are infrequent post-transplant biliary tract complications. Thirteen cases were discovered among 493 consecutive liver transplants (2.6%). Three (0.60%) were symptomatic (obstructive jaundice) while the other ten were found by systematically searching in the hilum in a series of 129 consecutive, resected grafts at retransplantation or autopsy (n = 54). Two types of cysts were detected: in eight grafts (1.6%), these were blind unilocular cavities with viscid mucous content, located adjacent to the biliary tract anastomoses. These had been inadvertently created as a result of the sequestered remnant cystic duct after cholecystectomies and biliary tract reconstructions, where a double-barreled common duct and long cystic duct had been present in the donor liver. These mucoceles ranged from 0.5 to 5.5 cm in diameter (median 1.7 cm). The three symptomatic cases were diagnosed by imaging techniques 3.5 years after transplantation; however, this type of cyst was found as early as the 2nd month post-transplantation when detected in lost liver grafts. Five livers (1%), lost between 5 months and 2.8 years post-transplantation, showed cystically dilated peribiliary glands, sometimes with multilocular, and occasionally multiple, cavities ranging from 0.5 to 2 cm in diameter (median 0.8 cm). This type of cyst was asymptomatic and located adjacent to the left, right, or common hepatic ducts. Threads were found near four cysts, suggesting that surgical injury may have been responsible for obstructing the neck of the glands. With the increasing number of long-term survivors of liver transplantation, unless preventive surgical methods are implemented, the number of symptomatic cysts of these origins can be expected to grow. Transplantation teams should, therefore, be aware of these potential causes of biliary tract complications.

Siringocistoadenoma papilífero de presentación atípica / Atypical presentation of syringocystoadenoma papilliferum

El siringocistoadenoma papilífero es un tumor anexial benigno derivado de las glándulas sudoríparas que en un 70-80 % de los pacientes se localiza en cabeza y cuello, generalmente en forma de pápula o nódulo solitario. Presentamos un caso de siringocistoadenoma papilífero inusual tanto por su localización, en un muslo, como por sus rasgos histológicos no estereotípicos de esta entidad

Syringocystoadenoma papilliferum is benign adnexal tumor derived from the sweat glands that is located in the head and neck in 70-80 % of patients, and commonly presents as a papule or a solitary nodule. We report a case of syringocystoadenoma papilliferum with an atypical presentation given its location in the thigh and the peculiar histologic features, unrepresentative of this entity

Citopatología de los carcinomas de mama / Cytopathology of the breast carcinomas

La principal indicación de la PAAF de mama es la existencia de una masa palpable y valorada conjuntamente con la exploración clínica y mamográfica (Triple test); su precisión diagnóstica es de un 99 por ciento. Los criterios citológicos que permiten establecer un diagnóstico de malignidad son: celularidad abundante (celularidad tumoral), grupos celulares tridimensionales y poco cohesivos, pleomorfismo celular, células aisladas con citoplasma, ausencia de núcleos bipolares desnudos y presencia de fondo sucio Aunque la mayoría de los carcinomas mamarios son de tipo ductal, existen parámetros citológicos que, aunque no siempre, permiten identificar subtipos específicos. El material obtenido con la PAAF permite la valoración de factores pronósticos, ya sea realizando técnicas inmunocitoquímicos para receptores hormonales o aplicando criterios que permiten valorar el grado nuclear. La PAAF de mama en las lesiones malignas aporta una sensibilidad del 93 por ciento y una especificidad del 94 por ciento (AU)