RESUMO
The pancreatic neuroendocrine tumors (PanNETs) most commonly present as solid neoplasms; however, very rarely, they may present primarily as cystic neoplasms. Most of the cystic PanNETs are non-secreting tumors, and the radiological features are not well defined. Hence pre-operative diagnosis is usually challenging and the tumors are misdiagnosed as mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, serous cystic neoplasms, solid pseudopapillary neoplasms, and non-neoplastic cysts. However, the management depends on the accurate diagnosis of these cystic lesions, which poses a dilemma. Herein, we report the case of a cystic PanNET in the tail of the pancreas, which was clinically and radiologically misdiagnosed as a mucinous cystic neoplasm. This case is reported to highlight this issue to the medical community regarding the diagnostic difficulty in such rare non-functioning pancreatic neuroendocrine tumors.
RESUMO
The pancreatic neuroendocrine tumors (PanNETs) most commonly present as solid neoplasms; however, very rarely, they may present primarily as cystic neoplasms. Most of the cystic PanNETs are non-secreting tumors, and the radiological features are not well defined. Hence pre-operative diagnosis is usually challenging and the tumors are misdiagnosed as mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, serous cystic neoplasms, solid pseudopapillary neoplasms, and non-neoplastic cysts. However, the management depends on the accurate diagnosis of these cystic lesions, which poses a dilemma. Herein, we report the case of a cystic PanNET in the tail of the pancreas, which was clinically and radiologically misdiagnosed as a mucinous cystic neoplasm. This case is reported to highlight this issue to the medical community regarding the diagnostic difficulty in such rare non-functioning pancreatic neuroendocrine tumors.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Cisto Pancreático/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Intraductais Pancreáticas/diagnóstico , Pâncreas/patologia , Neoplasias Císticas, Mucinosas e Serosas/diagnóstico , Doenças Raras , Erros de DiagnósticoRESUMO
INTRODUCTION: Hypersplenism in cirrhosis is not infrequent and may compromise with quality of life and therapy. Splenectomy is a therapeutic option, but information on results of splenectomy is scarce. MATERIAL AND METHODS: Consecutive patients with cirrhosis who underwent splenectomy between 2001-2010 were included in the study. Safety, efficacy of splenectomy and subsequent influence on therapy were evaluated. RESULTS: Thirty three patients (mean age 30.9 ± 11.6 years, 19 men, viral 48.5%, autoimmune 15.1%, cryptogenic 36.4%) underwent splenectomy. Twenty were Child's A, 13 Child's B. Twenty patients had > 6 months follow up. Common indications were inability to treat with interferon, transfusion-dependent anemia, recurrent mucosal bleeds, and large spleen compromising quality of life. Median hospital stay was 7 (4-24) days. There was no splenectomy related mortality. Twenty three (70%) patients had post-operative complications, most commonly infections. Two patients required percutaneous drainage of post-operative collections, and 1 needed re-exploration for intra-abdominal bleed. Subsequent to splenectomy platelet count (44,000 to 151,000/mm 3 , p < 0.01) and TLC (2,500 to 13,400/mm 3 , p < 0.01) had sustained increase in all patients except one. Five HCV cirrhotics completed interferon and ribavirin therapy, 4 achieved sustained viral response. The quality of life improved and there was no recurrence of infections, mucosal bleed or anemia requiring transfusions in any patient. In patients on long term follow up (median duration 27 months), the median Child's score improved from 6 at baseline to 5 at follow up (p < 0.05). CONCLUSIONS: Splenectomy was safe and effective in patients with cirrhosis, and improved therapeutic options as well as Child's score.