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.

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.

Poroid Neoplasms: A Clinicopathological Study of 13 Cases.

Introduction: Poroid neoplasms (PN) are a heterogeneous group of tumors deriving from sweat glands and folliculo-sebaceous units. Their histological classification and clinical features are challenging. Our aim was to report clinicopathological features of poroid neoplasms. Methods: It is a retrospective study including all cases of poroid neoplasms registered at our Pathology laboratory of Niamey National Hospital (February 2020-February 2024). Results: We registered 13 cases of benign poroid neoplasms: 10 classic poromas (CP) (76.9%), 2 poroid hidradenomas (PH) (15.4%) and 1 dermal duct tumor (DDT) (7.7%). Nine cases (69.2%) had preoperative clinical diagnosis of malignancy. The mean age was 41.1 years (range of 12-70 years) with a slight female predominance. Only 4/13 cases (30.8%) had classical palmoplantar locations. The tumors mean size was 3.7 cm (range of 0.4-8 cm). Clear cells were present in 7 cases (53.8%), apocrine ductal differentiation (mixed or pure) in 6 cases (46.2%), keratin horns in 2 cases (15.4%), squamous eddies in 6 cases (46.2%), melanin pigments in 1 case (7.7%) and sebaceous differentiation in 2 cases (15.4%). Conclusions: Unlike what is classically reported, our study shows that apocrine ductal differentiation, younger age and non-palmoplantar locations are common in poroid neoplasms.

Uterine carcinosarcomas: A case series of 9 cases from a low-income country.

RATIONALE: Uterine carcinosarcomas (UCS) are rare aggressive biphasic tumors classified as a subtype of high-grade uterine carcinomas. However, these tumors have particular histopathological features and clinical behavior with worse prognosis than high-grade uterine carcinomas. PATIENT CONCERNS: The incidence of UCS is increasing and more studies are required to elucidate their clinical and histopathological characteristics. Herein, we report clinicopathological features of 9 cases of UCS in a low-income country. DIAGNOSES: We retrospectively collected all cases of UCS at our Pathology Department over a period of 4 years. The diagnosis was performed on formalin-fixed, paraffin-embedded and hematoxylin and eosin-stained surgical specimens. INTERVENTIONS: Nine surgically treated cases of UCS have been registered, representing 12.67% of all uterine malignancies with a mean age of 58.88 years (range: 50-65 years). Abdominal pain and metrorrhagia were the main clinical presentations. The epithelial component of UCS was often a serous carcinoma (66.66%) and patients presented with large tumors (mean size of 9.24 cm, range of 5-19 cm), with advanced FIGO stages (stages III-IV) in 5/9 patients (55.55%). OUTCOMES: Follow-up data were available in 5/9 patients among which only 2 were alive 2 and 25 months after the surgical treatment (overall survival of 40%). LESSONS: UCS are rare and aggressive uterine tumors with very poor prognosis especially in low-income countries.

Proptosis Revealing a Rare Lacrimal Gland Tumor: A Case of Chondroid Syringoma in a 35-year-old Patient.

Lacrimal gland chondroid syringoma is a very rare tumor with classic clinico-radiological symptoms that should be familiar to clinicians for appropriate patients' management as the tumor has potential for recurrence and malignant transformation. We report herein a case of chondroid syringoma in a 35-year-old patient presenting with progressive painless proptosis for 2 years. He underwent complete surgical removal of the tumor, with subsequent clinical improvement of his symptoms.

Urinary bladder Schistosoma haematobium-related squamous cell carcinoma: a report of two fatal cases and literature review.

BACKGROUND: Schistosomiasis is still a public health issue in certain areas of developing countries (especially in sub-saharan Africa). Schistosoma haematobium is a proven carcinogenic agent that causes mainly bladder squamous cell carcinoma. This type of cancer has characteristic epidemiological, clinical and histopathological features with poor prognosis as compared to other urinary bladder cancers not associated with this parasite. CASES PRESENTATION: We report two fatal cases of advanced-stage bladder squamous cell carcinoma associated with Schistosoma haematobium in a sub-saharan developing African country (Niger), illustrating the devastating complications of this tropical neglected disease. The two cases were a 38-year-old woman and a 37-year-old male. They presented with chronic pelvic pain and hematuria. The clinical and radiological work-up revealed invasive urivary bladder tumor extended to the pelvis, that was histopathologically proven to be an invasive squamous cell carcinoma associated with Schistosoma haematobium. The two patients died shortly after the diagnosis before chemotherapy prescription. CONCLUSION: Schistosoma-associated bladder squamous cell carcinoma has characteristic features with dismal prognosis. Eradication of this parasite remains the only efficient way to prevent the devastating consequences of this particular cancer.