RESUMO
Gastric yolk sac tumors (YSTs) are very rare. Only seven cases of pure YST of the stomach, with a median age of 65 years, and five cases of YST combined with adenocarcinoma have been reported. We report a 3-year-old boy who presented to us with recurrent episodes of melena and anemia of 2 months' duration. Upper gastrointestinal endoscopy revealed a deeply excavated ulcer on the lesser curvature, 4 cm from the gastroesophageal junction. Computed tomography (CT) abdomen revealed large heterogeneously enhancing soft-tissue mass lesion in the lesser sac with indistinct fat planes with the lesser curvature of the stomach with multiple peripherally enhancing lesions in both lobes of liver and omentum, suggestive of metastasis. Serum alpha-fetoprotein (AFP) was 21,000 ng/ml. Tru-cut biopsy from the mass was suggestive of YST. Following three courses of chemotherapy based on Cisplatin, Etoposide and Bleomycin (PEB), sleeve gastrectomy with excision of the mass with omentectomy with biopsy of the liver nodules with a sampling of perigastric lymph nodes was done. Histopathology revealed only necrotic tissue with no evidence of residual malignancy. Three-month follow-up CT revealed no residual mass with AFP of 3 ng/ml.
Assuntos
Duodenopatias/etiologia , Corpos Estranhos/etiologia , Fístula Intestinal/etiologia , Doenças do Jejuno/etiologia , Imãs , Jogos e Brinquedos , Pré-Escolar , Duodenopatias/diagnóstico , Duodenopatias/cirurgia , Corpos Estranhos/diagnóstico , Corpos Estranhos/cirurgia , Humanos , Fístula Intestinal/diagnóstico , Fístula Intestinal/cirurgia , Doenças do Jejuno/diagnóstico , Doenças do Jejuno/cirurgia , MasculinoRESUMO
All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder. In the presence of above 'red flag' signs, there has been much debate on diagnostic accuracy of percutaneous liver biopsy (PLB) vs. hepatobiliary scintigraphy (HBS) to substantiate or exclude BA. Recent guidelines suggest a shift towards PLB (91.6% overall diagnostic accuracy) as the diagnostic cornerstone with key differentiating feature being 'bile ductular proliferation'. HBS has a high (98.7%) sensitivity but low specificity (37-74%) with an overall diagnostic accuracy of 67% for BA. Severe hepatocellular disease without anatomic obstruction would also have a non-excretory scan. Thus, while excretory HBS excludes BA, non-excretion does not confirm BA. Hence, diagnostic algorithms relying on non-excretory HBS as the primary standalone benchmark for surgical exploration would be mired by a high negative laparotomy rate revealing a normal peroperative cholangiogram (POC). However, an excretory HBS obviates need for laparotomy in case of equivocal stool color or PLB. A POC continues to be the ultimate gold standard. Hence, with high index of clinical suspicion but equivocal ultrasonography or PLB and a non-excretory HBS, the baby should not be denied a POC within time frame crucial for successful hepatoportoenterostomy.