Patient: Male, 19-year-old Final Diagnosis: Splenic hamartoma Symptoms: Abdominal pain Medication:— Clinical Procedure: Laparoscopic splenectomy Specialty: Surgery Objective: Rare disease Background: Splenic hamartoma (SH) is a benign vascular lesion, usually found incidentally on abdominal images or at autopsy. Only around 200 cases have been reported since 1861, when SH was first described by Rokitansky. Although it is very rare, it is important to be familiar with it, as it may be a diagnostic challenge to distinguish SH from other mass lesions of the spleen based solely on preoperative investigations. Case Report: We describe a case of symptomatic, isolated, single splenic hamartoma in a 19-year-old, otherwise healthy young man who presented with upper abdominal pain, nausea, and vomiting for a few months. The examination was unremarkable. The patient has been previously evaluated with abdominal ultrasonography, which found a suspicious splenic hyperechoic lesion. Computed tomography revealed a heterogeneous 5×7 cm enhancing lesion in the spleen, concerning for splenic hamartoma. The patient underwent laparoscopic splenectomy and recovered well. The histopathology examination confirmed the diagnosis of splenic hamartoma. Conclusions: Splenic hamartoma is a rare benign vascular lesion of debated etiology. Most cases are asymptomatic and are found incidentally on images, in splenectomies performed for other reasons, or at autopsy. Radiologic findings may suggest the diagnosis and new modalities have shown accuracy in distinguishing splenic hamartomas. However, resection with formal or partial splenectomy is usually still needed since the differential diagnosis is wide, from benign to aggressive lesions, and histopathology remains the criterion standard for diagnosis. Given its benign nature, we found no cases of recurrence or metastasis in the literature.
Kikuchi–Fujimoto disease (KFD) is a rare benign and self-limiting syndrome. We aim to review cases of KFD at our institution as a rare illness in the Arab ethnic descent and to analyse reports from most countries in the East Mediterranean zone. This is a retrospective study in which the histopathology database was searched for the diagnosis of KFD. A full review of KFD patients’ medical records was done. Data regarding demographic features, clinical presentation, laboratory findings, comorbidities, and management protocols were obtained. Published KFD cases from east Mediterranean countries were discussed and compared to other parts of the world. Out of 1968 lymph node biopsies studied, 11 (0.6%) cases of KFD were identified. The mean age of patients with KFD was 32 years (4–59). 73% (8/11) were females. The disease was self-limiting in 5 patients (45%); corticosteroid therapy was needed in 4 patients (34%). One patient was treated with methotrexate and one with antibiotics. One patient died as a consequence of lymphoma. Jordanians and Mediterranean populations, especially those of Arab ethnic background, seem to have low rates of KFD. The genetic susceptibility theory may help to explain the significantly higher disease prevalence among East Asians. Early diagnosis of KFD—although challenging—is essential to reduce the morbidity related to this illness.
Background Kikuchi-Fujimoto disease (KFD) is a rare benign and self-limiting syndrome. We aim to review cases of KFD at our institution as a rare illness in the Arab ethnic descent, and to analyze reports from most countries in the east Mediterranean zone. Methods This is a retrospective study in which the histopathology database was searched for the diagnosis of KFD. A full review of KFD patients’ medical records was done. Data regarding demographic features, clinical presentation, laboratory findings, comorbidities and management protocols were obtained. Published KFD cases from east Mediterranean countries were discussed and compared to other parts of the world. Results Out of 1968 lymph node biopsies studied, 11 (0.6%) cases of KFD were identified. Mean age of patients with KFD was 32 years (4-59). 73% (8/11) were females. The disease was self-limiting in 5 patients (45%), corticosteroid therapy was needed in 4 patients (34%), one patient was treated with methotrexate and one with antibiotics. One patient died as a consequence of lymphoma. Conclusion KFD may still be under diagnosed in our part of the world, likely due to the non-specific mixed symptoms at presentation as well as to the lack of awareness among general practitioners and health care providers. Early diagnosis and treatment is essential to reduce associated morbidity.
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