RESUMO
BACKGROUND: Retrorectal hamartoma, also known as tailgut cyst, is a rare tumor that arises from remnants of the embryonic postanal gut. The tumor occurs in the retrorectal space and may undergo malignant degeneration. METHODS: The clinical information on a single patient with pseudomyxoma peritonei associated with a tailgut cyst was reviewed and a literature review of the subject performed. RESULTS: In this patient there was malignant degeneration of a tailgut cyst localized to the pelvis. Twenty months later a local recurrence in the pelvis and pseudomyxoma peritonei widely distributed in the abdomen were successfully treated by cytoreductive surgery and perioperative chemotherapy. CONCLUSION; Tailgut cyst may, if resected with tumor spillage, result in pseudomyxoma peritonei. Our treatment--which has maintained a disease-free status for one year--was cytoreductive surgery plus perioperative chemotherapy.
Assuntos
Adenocarcinoma Mucinoso/complicações , Hamartoma/complicações , Neoplasias Primárias Múltiplas/complicações , Neoplasias Peritoneais/complicações , Pseudomixoma Peritoneal/complicações , Doenças Retais/complicações , Adenocarcinoma Mucinoso/patologia , Adulto , Antineoplásicos/uso terapêutico , Terapia Combinada , Procedimentos Cirúrgicos do Sistema Digestório , Feminino , Hamartoma/patologia , Hamartoma/terapia , Humanos , Neoplasias Primárias Múltiplas/patologia , Neoplasias Peritoneais/patologia , Neoplasias Peritoneais/terapia , Pseudomixoma Peritoneal/patologia , Pseudomixoma Peritoneal/terapia , Doenças Retais/patologia , Doenças Retais/terapia , Tomografia Computadorizada por Raios XRESUMO
Hepatic amyloid deposition in the form of globular inclusions is a rare occurrence. Only 24 such cases have been reported to date. Its clinical and pathological significance are undefined. Unawareness of such a pattern can cause diagnostic confusion. We herein describe a case of globular hepatic amyloid in a patient with a B-cell lymphoma and chronic hepatitis C. The findings in our case support the literature data in that (1) it is often an incidental finding during workup for other coexisting conditions and (2) its morphology is peculiar but can be recognized with ease if one is aware of its existence. Our case also provides new insights into this condition. First, it represents the first nonautopsy case to demonstrate that globular hepatic amyloid is an indication of systemic amyloidosis, thus emphasizing the clinical importance of the recognition of this condition. Second, in our case, there was a coexisting B-cell lymphoma, the constituent cells of which showed immunoglobulin lambda light chain restriction, and patient's serum lambda light chain was elevated. However, light chain restriction was not demonstrated in the globular inclusions, and there was no evidence of monoclonal gammopathy by serum electrophoresis. Whether immunoglobulin light-chain abnormality played a causal role in this condition is to be determined.