A 19-year old male Caucasian patient visited the emergency department of our hospital because of melena. He was previously healthy and used no medication but had noticed black stools one week before his visit. There had been no abdominal discomfort, nausea or vomiting. None of his family members had gastrointestinal diseases, and he used no alcohol, did not smoke or use elicit drugs. His occupation is unknown. On physical examination the abdomen was nontender and without palpable masses. Rectal examination revealed black stools. His weight was 62 kg. and his length 1,87 m. Laboratory tests showed an iron deficiency anemia with a hemoglobin concentration of 4.7 mmol/L (normal > 8,5 mmol/L) and a serum ferritin concentration of 4 μg/L (normal > 30 μg/L). Liver function tests, serum creatinine, albumin, CRP and glucose were all normal. Duodenoscopy showed a normal esophageal, gastric and duodenal mucosa without evidence of recent bleeding. A Meckel scan showed no evidence for a Meckel's diverticulum. A small bowel series however, demonstrated a large polyp in the proximal jejunum and possibly a smaller polyp in the distal jejunum (Figure ).\nAs this polyp was the probable cause of the patient's bleeding, a laparotomy with peroperative small bowel endoscopy was performed. Approximately 50 cm beyond Treitz' ligament, a multilobular, 40 mm large polyp was seen, which was subsequently surgically resected (Figure ). There were no other small bowel polyps; the suspected smaller polyp seen on the small bowel series was therefore most likely an artefact.\nPathologic examination of this polyp demonstrated a hamartomatous polyp with arborization of smooth muscle compatible with a PJP (Figure ). No signs of dysplasia were seen and the surrounding tissue had a normal histological appearance. Immunohistochemistry was negative for p53, focally positive for cyclin D1 and the basal parts of the cryps were positive for Ki67/MIB1.\nThe postoperative course has been unremarkable and no further gastrointestinal bleeding has occurred. Total colonoscopy did not show any additional polyps. A repeated, extensive family history, performed by a clinical genetics consultant, did not reveal clues (including pigmentation, nasal or gastrointestinal polyps or typical secondary tumors) to the occurrence of PJS in family members.
In 2003, a 40-year-old woman consulted a doctor because of feeling a palpable mass in the right breast and bleeding discharge from both breasts. Mammography was taken and revealed bilateral mass with infiltrating margins. Excisional biopsy was performed on both breasts. In histopatholgical examination, grossly; the right side showed a nonencapsulated mass with 5.0 × 4.0 × 1.0 cm size as well as relatively dense consistency and the left one showed the same mass with 6.0 × 3.0 × 2.0 cm size. In microscopic examination it was showed carcinoma in atypical sclerosing papilloma of both breasts [].\nOne month later bilateral modified radical mastectomy and axillary lymph nodes dissection were performed. In microscopic examination there was no residual of primary tumor and the only abnormality is atypical ductal hyperplasia. Lymph-nodes, skin, and nipple were free. Three months later a colonoscopy was performed and multiple polyps were found in the large bowel. Biopsy was taken and the result was hamartomatous polyp [].\nTwo years later in 2005, she was referred to the hospital again because of abnormal uterine bleeding (menometrorrhagia). Pelvic ultrasound showed increased endometrial thickness with normal adnexal regions. Bilateral salpingo-oophorectomy was performed. In histopathological examination, grossly, the uterus was measured 10.0 × 8.0 × 3.0 cm. The right ovary was measured 4.0 × 2.0 × 1.0 cm and the left one 4.0 × 3.0 × 1.0 cm. The cut surface of both ovaries showed nonhomogenous appearance with no obvious lesion. In microscopic examination, both ovaries showed sharply circumscribed rounded epithelial nests composed of ring-shaped tubules encircling the hyalinized basement membrane-like material. The nests have two basic patterns: The simple pattern is that of a single tubule encircling a central rounded hyaline mass, and the complex pattern is characterized by communicating tubules encircling multiple hyaline masses. These features are compatible with sex cord tumor with annular tubules that are usually associated with Peutz–Jeghers syndrome []. Except simple hyperplasia of endometrium; no any pathologic feature was found in the endometrium.