Transformación nodular angiomatoide esclerosante del bazo: reporte de caso / Sclerosing angiomatoid nodular transformation of the spleen: a case report

La transformación nodular angiomatoide esclerosante es una patología vascular benigna del bazo, desarrollada a partir de la pulpa roja, de etiología desconocida. Se postula que puede estar relacionada con la enfermedad por inmunoglobulina 4 y la infección por el virus de Epstein-Barr. La mayoría de los casos son asintomáticos, constituyendo hallazgos incidentales en estudios por imágenes. Presentamos el caso de un paciente masculino de 41 años con antecedentes de tiroidectomía por carcinoma papilar que consulta por fiebre. Recibió tratamiento sintomático y se realizó tomografía computarizada de abdomen por síntomas abdominales inespecíficos. La tomografía evidenció una imagen de aspecto sólido, con tenue realce periférico con el contraste que mide 62 por 52 por 51 milímetros en el polo inferior del bazo. Se realizó esplenectomía que midió 14 por 11 por 4 centímetros y pesó 284 gramos. Se identificó una formación nodular sólida, bien delimitada, con área central de aspecto fibroso, con tractos blanquecinos que delimitan áreas violáceas. La microscopía presentó nódulos coalescentes redondeados de aspecto angiomatoide, con proliferación vascular revestida por células endoteliales sin atipia, entremezclados con células ahusadas, infiltrado de linfocitos y macrófagos. El estroma entre los nódulos mostró proliferación miofibroblástica con linfocitos, plasmocitos y siderófagos. Inmunohistoquímica tuvo marcación positiva en los vasos para CD34 y CD31, sectores positivos para CD8 y negativos para CD34. Una célula positiva para inmunoglobulina 4 (IgG4) por campo de gran aumento. El estudio para Epstein-Barr por reacción en cadena de la polimerasa fue negativo. Para el diagnóstico los estudios de imagen son inespecíficos, por lo que la confirmación diagnóstica la da el estudio histopatológico. La esplenectomía es curativa sin casos reportados hasta la actualidad de transformación maligna o recidiva. No se conocen factores de riesgo y no se han comprobado factores desencadenantes, excepto la asociación de casos con IgG4 y virus de Ebstein-Barr. Por ser una entidad patológica recientemente descrita es necesario recopilar series grandes y revisar nuestros archivos, reevaluando algunos de sus diagnósticos diferenciales para lograr una mejor comprensión de la misma.

Sclerosing angiomatoid nodular transformation is a benign vascular pathology of the spleen, developed from the red pulp, of unknown etiology; it is postulated that it may be related to IgG4 disease and Epstein-Barr virus infection. Most cases are asymptomatic, constituting incidental findings in imaging studies. We present a 41-year-old male patient with a history of thyroidectomy for papillary carcinoma who consulted for fever, received symptomatic treatment and performed a computed tomography of the abdomen for nonspecific abdominal symptoms, the same evidence in the lower pole of the spleen a solid-looking image with faint Peripheral enhancement with contrast, measures 62x 52x51 mm. A splenectomy measuring 14x 11x4 cm and weighing 284 grams was performed, identifying a solid, well-defined nodular formation, with a central fibrous-looking area, with whitish tracts that delimited purplish areas. Microscopy: rounded angiomatoid-like coalescing nodules, with vascular proliferation lined by endothelial cells without atypia, interspersed with spindle cells, infiltrated by lymphocytes and macrophages. The stroma between the nodules shows myofibroblastic proliferation with lymphocytes, plasma cells, and siderophages. Immunohistochemistry: positive labeling in vessels for CD34 and CD31, positive sectors for CD8 and negative for CD34. One IgG4 positive cell per high power field. The study for Epstein-Barr by Polymesara Chain Reaction was negative. For the diagnosis, the imaging studies are nonspecific, so the diagnostic confirmation is given by the histopathological study. Splenectomy is curative with no reported cases of malignant transformation or recurrence to date. There are no known risk factors and no triggering factors have been proven, except the association of cases with IgG4 and Ebstein-Barr virus. As it is a recently described pathological entity, it is necessary to collect large series and review our files, reevaluating some of its differential diagnoses to achieve a better understanding of it

Hemoptysis and Hemoperitoneum due to Metastatic Gestational Choriocarcinma: Bronchial Artery Embolization and Superselective Splenic Artery Embolization: A Case Report

Gestational choriocarcinoma is easily disseminated hematogenously and its hypervascular nature places the patient at risk of significant hemorrhage both at the sites of metastatic lesions and in the uterus. In addition, its tends to give rise to pseudoaneurysm formation. Treatment of the condition by percutaneous embolization has been reported in several published articles, and hemoperitoneum secondary to rupture of splenic metastasis of gestational choriocarcinoma has also been reported, as has angiographic embolization. Hemoptysis resulting from pulmonary metastasis and treatment by means of embolization of the bronchial artery have not been reported, however. In this article, we describe a case of hemoptysis and hemoperitoneum due to pulmonary and splenic metastasis of gestational choriocarcinoma. Treatment of the condition involved embolization of the bronchial artery and superselective embolization of the splenic artery.

Space occupying lesion of the spleen / 中国普通外科杂志

Objective To summarize the experience in diagnosis and management for the space occupying lesion of spleen. Method The clinical data of 29 cases treated by surgery were retrospectively analyzed. Results There were 15 patients with benign masses including 7 hamartomas, 5 hemangiomas, 1 pseudocyst, 2 tuberculoses of the spleen, and 14 with malignant tumors including 9 lymphomas, 3 angiosarcomas, 2 metastatic tumors in the spleen. Splenectomy was performed in all patients. All patients with benign masses survived except 2 patients lost follow up and 1 coexisting with hepataocellular carcinoma died half a year after the operation. Twelve of 14 patients with malignant tumor were followed up.Of them, 5 patients survived more than 5 years and 2 were alive 1 and 3 years after the operation respectively; 5 patients died 6 months to 4 years after the operation. Conclusions Ultrasonography and CT or MRI are the main means of diagnosis for the space occupying lesion of spleen.It is difficalt to make diagnosis of the splenic tuberculosis before operation.Splenectomy is a primary procedure of surgery.

Imaging of Nontraumatic Benign Splenic Lesions

The spleen is one of the largest organ in the reticuloendothelial system and plays an important role in theac-tivation of immune response. It is the organ most commonly injured after blunt abdominal trauma, and malig-nantlesions such as lymphoma, or these due to metastasis, occur not infrequently. Even so, it is ignored even inabdominal ultrasonography. Some benign splenic lesions, however can cause severe symptoms and result in high mortality, and their accurate diagnosis is therefore essential. This study describes the imaging findings andhistopathologic features of various nontraumatic benign splenic lesions.

Splenic Space Occupying Losion Observed in The Dual-Phase Bolus CT File

PURPOSE: To evaluate the dual-phase bolus CT findings and clinical significance of splenic S. O. L.(SpaceOccupying Lesion) basically included in abdominal CT but previously not considered important. MATERIALS AND METHODS: We retrospectively reviewed 64 splenic S.O.L.'s(0.94%) detected among 6842 patients who underwentdual-phase rapid sequence abdominal CT. Diffuse splenomegaly and heterogeneous enhancement during the arterialphase were excluded. Fifty-eight of 64 splenic S. O. L. cases were confirmed by surgery, biopsy, radiologic studyand follow up. RESULTS: In only eight patients(12.5%) were symptoms localized to the left upper quadrant. Therewere 21 cases of inflammatory and ischemic diseases(36.2%), 19 malignant tumors(32.8%), 12 benign cysts(20.7%),four benign tumors(6.9%), one metabolic disease and one hematoma(each 1.7%) ; six cases were not confirmed and inthese, S.O.L. was also found at other sites. Escept in the case of benign cysts and tumors, the early phase wasbetter than the late for the detection of S.O.L., though both were good for diagnosis. Among 21 inflammatory andischemic lesions there were eight cases of portal hypertensions, seven of pancreatitis, and one of tuberculosis ;five were due to other cauese. Among 19 patients with malignant tumors, metastases were most common(11 cases).Cases involving malignancy involved four lymphomas, two cases of leukemias and two angiosarcomas, which togetherrepresented only primary splenic malignancy. Two epidermoid and ten simple cysts were benign, while benign tumorsincluded three hemangiomas and one lymphangiomatosis ; these were difficult to differentiate from angiosarcoma.Gaucher's disease showed multiple low density lesions in the enlarged spleen and one hematoma was also present. CONCLUSION: Splenic S.O.L.'s are very rare and clinical symptoms directly related to splenic mass are uncommon.Benign and secondary lesions are more common than malignant and primary lesions, and cysts are also much rarerthan any other solid organs. Dual-phase CT especially during the early phase, is currently the modality of choicefor the evaluation of splenic S.O.L.