Peutz Jeghers Syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and hamartomatous polyps throughout the gastrointestinal tract. Pigmented macules appear especially on oral mucosa, lips and fingers. Hamartomatous polyps are found predominantly at the small intestine and colon lesser extent 1 and can cause bleeding and abdominal pain secondary to intussusception, intestinal obstruction and infarction. Approximately 80% of patients with PJS have mutations in threonine kinase 11 (STK11) located in 19p13.3 chromosome 1,2 . This acts as a tumor suppressor gen, leading its mutation to increased risk of cancer at an early age 3 . Most commonly reported cancers are luminal gastrointestinal and breast. Testicular tumors are less known, but may be present at early age 2 and course with estrogenic manifestations in most of cases.We report a case of PJS with intratubular large cell hyalinizing Sertoli Cell (LCHSC) tumor of the test is diagnosed during the follow-up of the patient.
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