PRIMARY NEUROENDOCRINE TUMOR OF THE SPLEEN: CASE REPORT

Case Report: Male, 39 years old, complaining of progressive and compressive abdominal pain in the left hypochondrium, for 2 years ago and worsening for the last 6 months ago, due to enlargement of the abdominal region causing dyspnea. Chronic renal patient on dialysis, hypertensive and with previous bone marrow biopsy which suggest hypercellularity for the age, with alterations pointing to chronic myeloproliferative disorder. Laboratory tests showed pancytopenia and significant renal changes. Abdominal ultrasound (USG) showed accentuated splenomegaly, enlarged liver associated with portal and splenic vein ectasia and presence of free fluid in the upper abdomen. Abdominal tomography confirmed findings described in the US. After hematological evaluation, it was decided to perform total open splenectomy with pre and postoperative prophylaxis for encapsulated germs. The anatomopathological analysis showed solid epithelial neoplasia involving periosplenic ductal tissue and absence ofperi-spleniclymphnodedisease. Immunohistochemistry confirmed the diagnosis of neoplasia, positive for the synaptophysine and chromogranin antibodies indicating the neuroendocrine differentiation of neoplastic cells, being low grade neuroendocrine neoplasia (NET 1). Discussion: Neuroendocrine Tumors (NET) are so-called because they characterize a group of rare tumors originating from hormone-producing cells of the endocrine system. Determined to be heterogeneous and slow growing, they affect almost all organs, predominating in the gastrointestinal tract (60%) and respiratory tract (25%). INCA estimates 5.25 new cases per 100 thousand inhabitants, with prevalence in the sixth and seventh decade of life. NET may be an incidental finding, as symptoms are nonspecific, depending on their location and hormone production. Carcinoid syndrome may occur, characterized by facial flushing, abdominal pain, and diarrhea, usually with liver metastasis. To decide on the treatment, imaging and biopsy are performed, which will confirm the diagnosis by identifying if there is local lymphadenopathy and determining the proliferation index (Ki-67). Final considerations:Thus, it was defined as primary spleen NET, as there are no cases described in the literature that support this report. The patient of the case in question is being followed up with no signs of return of tumor activity.

 

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Publication History

Article published online:
23 October 2019