Appendiceal carcinoid in a pediatric patient with Peutz-Jeghers syndrome: A case report and comprehensive literature review.

Peutz-Jeghers syndrome (PJS), a rare autosomal dominant disorder, is characterized by mucocutaneous pigmentations, hamartomatous polyps in the gastrointestinal tract, and a high risk of developing various malignancies. To the best of our knowledge, only 1 case of appendiceal carcinoid associated with PJS has been previously reported in the pediatric population. We report a 7-year-old girl who was admitted for severe, intermittent abdominal pain and cramps, nausea, and vomiting. Multiple brown melanotic macules on the lips, buccal mucosa, and the tongue were noted. A plain abdominal X-ray in a standing position revealed dilated intestinal loops with multiple air-fluid levels. A computed tomography scan of the abdomen showing a "coffee bean" appearance of the jejunal loop with a transition point to the duodenal loop. Axial-contrast-enhanced computed tomography scan of the abdomen showing dilated jejunum loops, filled with fluid with the swirled appearance of mesentery typical for volvulus. The diagnosis of PJS was based on clinical findings along with the histopathologic confirmation of the hamartomatous polyps. An emergency laparotomy was performed, revealing a jejunojejunal intussusception starting 40 cm from the duodenojejunal flexure. Jejunotomy revealed that a lead-point intussusception was a necrotic hamartomatous polyp. After resecting the involved jejunal necrotic segment, including the polyp, end-to-end jejuno-jejunal anastomosis was performed. Further exploration revealed the presence of a jejunal mass 80 cm from the duodenojejunal flexure identified as another hamartomatous pedunculated polyp. The polyp was resected, and the enterotomy was then closed transversely. The grossly normal appendix was also removed. Clinical findings along with the histopathologically confirmed hamartomatous polyps were consistent with PJS. An appendiceal carcinoid (well-differentiated neuroendocrine tumor, European Neuroendocrine Tumor Society stage pT2) was incidentally detected during histological examination of the appendix. The patient and parents were counseled accordingly, focusing on active surveillance and control of symptoms. Two additional hamartomatous polyps (gastric and jejunal) were detected endoscopically and resected in the fourth postoperative week. A regular, 1-year follow-up and surveillance revealed no complications or recurrences. Unusual neoplasms can occasionally be encountered in well-defined syndromes such as PJS. Therefore, active follow-up and surveillance are mandatory for all patients with PJS

Advanced adult granulosa cell tumor with prominent fibromatous stroma: A case report

To the Editor, Adult granulosa cell tumor (AGCT) is the most common sex cord–stromal tumor and accounts for about 1% of all ovarian neoplasms.1 It is derived from granulosa cells of the ovarian follicle that are responsible for estradiol production and are admixed with a variable population of fibroblasts or theca cells. AGCT usually affects perimenopausal women (mean age ∼50–55 years).2 The patients present with abdominal pain or estrogenic manifestations (e.g., uterine bleeding).2 AGCTs tend to be unilateral, with solid and/or cystic growth patterns. Most patients present with stage I disease, associated with a 10-year survival rate of 90–95% and a recurrence rate of 10–15%; the overall recurrence rate for all stages combined is 20–30%. Extraovarian spread includes the peritoneum and omentum and rarely to the liver, lungs or bone.2,3 Tumors have many histologic patterns, while their stroma varies from scanty to abundant, which can be fibromatous or thecomatous.1,4All performed procedures were done per the ethical standards of the 1964 Helsinki declaration. Institutional review board (IRB) approval was not requested for the case report.Scopu

A Giant Polypoid Gastric Heterotopia of the Ileum as a Cause of Intussusception in an Adolescent.

Histopathological examination confirmed a well-demarcated polyp, lined by a foveolar-type epithelium, with all the elements found in the gastric mucosa, and a well-developed gastric gland. No dysplasia or malignancy was identified within the gastric heterotopia. On the basis of the radiological, intraoperative, and histopathological features, a giant polypoid gastric heterotopia was diagnosed. A second opinion was sought from an expert gastrointestinal pathologist who concurred with the final diagnosi

Breast lump as the initial presentation of metastatic uterine leiomyosarcoma: a case report and comprehensive literature review

Uterine leiomyosarcoma (uLMS) is a rare but aggressive cancer with a high metastatic potential and an unfavo-rable prognosis. A 54-year-old woman with a history of uterine fibroids clinically presented with a painless, palpa-ble left breast mass measuring 20 mm. A core biopsy of the breast mass demonstrated a cellular spindle cell neo-plasm (a potentially malignant smooth muscle neoplasm; B4). A wide local breast-mass excision was performed, re-vealing grade-2 leiomyosarcoma. A re-review of the uterine fibroids revealed that the largest one (200 x 130 mm), initially diagnosed as symplastic leiomyoma, was morpho-logically identical to the breast lesion. Additional diagnostic work-up revealed multiple liver and pulmonary metastases with a suspected metastatic sclerotic lesion in the L3 projection. The patient was subsequently treated with chemotherapy protocol for metastatic uLMS. The latest fol-low-up in September 2023 confirmed stable disease. This case highlights the importance of considering unusual metastatic patterns when evaluating breast masses, par-ticularly in patients with a history of non-specific uterine conditions. Comprehensive diagnostic work-up, including imaging and histopathologic examinations, is crucial for an accurate diagnosis of uLMS and appropriate treatment se-lection. Further studies are needed to better understand the underlying mechanisms and optimal management strategies for metastatic uLMS.Scopu

Large uterine juvenile cystic adenomyoma in an adolescent

Juvenile cystic adenomyoma (JCA) is a rare uterine pathology with <40 cases reported in the current literature since 1996 when Tamura described it. We report a 13-year-old girl with a history of chronic pelvic pain and dysmenorrhea for 12 months. After diagnostic evaluation and identification of the cystic structure (∼6 cm) within the myometrium, fertility-sparing surgery was successfully performed. Histopathological examination of the uterine cyst was consistent with the diagnosis of JCA. The postoperative course was uneventful, and the patient was disease-free three years after surgery. JCA is a rare condition but should be considered in the differential diagnosis in adolescents with moderate to severe dysmenorrhea. Despite diagnostic advances, the awareness of the disorder remains low. Thus, our case report aims to increase awareness of this rare pathology with unclear etiology.The article-processing fee was covered by the Qatar National Library (QNL)