Sporadic Zollinger-Ellison syndrome in a patient with isolated mesenteric gastrinoma.

INTRODUCTION: Zollinger-Ellison syndrome (ZES) is due to a gastrin-producing neuroendocrine tumor (gastrinoma) with subsequent gastric acid hypersecretion, gastroesophageal reflux disease (GERD), peptic ulcers, and chronic diarrhea. CASE PRESENTATION: A 40-year old patient presented with epigastric pain associated with diarrhea evolving for 10 years, he has been treated by acid-suppressive medications without substantial clinical improvement. Gastro-duodenal endoscopy was performed with gastric biopsies that showed peptic ulcerations, a serum gastrin assay that showed high levels exceeding 10 times the upper limit of the normal range. The abdominal contrast-enhanced computed tomography scan (CT-Scan) showed a solid-cystic mass with contrast enhancement in the right colon mesentery. The histopathological analysis of the resected mass showed a well-differentiated neuroendocrine tumor, and the diagnosis of a sporadic Zollinger-Ellison syndrome (ZES) was disclosed. Eighteen months after the surgical treatment the patient has no symptoms of the disease. CLINICAL DISCUSSION: Patients with ZES present often with long-standing clinical symptoms with delayed diagnosis. The surgical resection of the causative gastrinoma remains the most effective therapeutic treatment. CONCLUSION: Patients with refractory chronic diarrhea and epigastric pain should be suspected of having ZES in order to benefit from adequate clinical management.

Splenic lymphangiomas as a common indication for splenectomy: a case series with literature review.

BACKGROUND: Splenic lymphangiomas (SL) are very rare benign cystic lesions found in pediatric population. Their occurrence in adults is exceptional. Splenectomy is the common management of splenic lesions for diagnostic and/or therapeutic purpose. Our aim is to report additional cases of SL diagnosed on splenectomy specimens at our Pathology laboratory with literature review. METHODS: This is a retrospective study including all cases of splenectomy recorded at our Pathology laboratory (June 2020-August 2022). We performed a comparison of clinicopathological features between patients with SL and those with other benign splenic diseases. RESULTS: Sixteen cases of splenectomy were included. The mean age was 30.25 years (range of 6-70 years). The final histopathological diagnoses were congestive spleens in all cases of sickle cell disease (SCD) (5/16 patients, 31.25%), splenic cystic lymphangiomas (4/16 patients, 25%), capsular splenic infiltration by gastric and colic cancers (3/16 cases, 18.75%), splenic abscess (2/16 cases, 12.5%) and splenic rupture with subcapsular hematoma (1/16 patients, 6.25%). 12/16 patients (75%) had benign splenic conditions (4/12 with SL, 5/12 with SCD, 2/12 with abscess and 1/12 with splenic trauma). Patients with SL were older than those with other benign splenic conditions (mean age of 28.27 years versus 20.87 years). Also patients with SL presented with massive splenomegaly (mean splenic weight of 1675 g versus 418.75 g, mean splenic size of 19.62 cm versus 14.63 cm). Open surgery was performed in 15/16 patients (93.75%). CONCLUSION: Unlike previous studies, our series shows that SL are a common indication for splenectomy and occur in older patients with massive cystic splenomegaly. Open splenectomy is still an usual surgical practice in our country.

Aggressive angiomyxoma as a rare cause of scrotum enlargement in a 10-month-old boy: a case report.

BACKGROUND: Aggressive angiomyxoma (AAM) is a locally infiltrative mesenchymal tumour that most commonly affects the pelvis and/or perineum in adult women. AAM is very rare in males, especially in infancy. CASE PRESENTATION: A 10-month-old fulani (African) male infant was referred to our department for a large painless mass in the right testicule. The mass was detected during the neonatal period and gradually increased in size. Ultrasound examination revealed a large heterogeneous lesion; computed tomography results led to the conclusion that the mass was a mesenteric hernia. An inguinal and scrotal surgical approach was adopted. Exploratory surgery found a normal right testicle displaced upwardly and a large scrotal mass. Radical excision of the mass and orchidopexy were performed. Subsequent histology and immunohistochemstry studies indicated that the mass was a scrotal angiomyxoma. The postoperative course was uneventful. No recurrence occurred during the 6-month follow-up. CONCLUSION: To the best of our knowledge, this is the youngest patient with AAM reported to date. Angiomyxoma should be included in the differential diagnosis of scrotal masses, for which radical excision is justifiable to prevent recurrence.