A 22-year-old male patient was referred to us for evaluation of multiple pigmented lesions over his mouth, present since childhood. Since the age of 9 year, there was a history of occasional hematochezia. However, there was no history of melena. The parents had taken him to a local physician, and the condition was managed with antihelminthic drugs, but the problem used to recur. There was no history of weight loss or any other systemic complaints. Five days back, he had presented to the general emergency with complaints of nonpassage of stool and flatus, pain, and distension of abdomen and vomiting. He had tachycardia, tachypnea, dehydration, hypotension, and distended rigid abdomen. Per rectal examination revealed empty rectum with blood mixed mucus. Straight X-ray abdomen showed dilated bowel loops, multiple air-fluid levels, and the absence of gas in colon and rectum []. Diagnosis of small bowel obstruction was made, and the patient was resuscitated. Hematological profiles and serum electrolytes were within the normal limits. Exploratory laparotomy was done. On exploration, ileocecal intussusception was found with a short segment of gangrenous ileum which was managed with limited ileocecal resection []. Numerous polyps were found along the whole length of the small and large gut. Proximal ileostomy with distal mucous fistula was made. A polyp was found to be the apex of the intussusception, which was histologically found to be a hamartomatous polyp [].\nDuring follow-up, upper gastrointestinal endoscopy and colonoscopy were done which revealed the presence of polyps from the gastric fundus to the rectum. Biopsy from those polyps confirmed a hamartomatous pathology []. Besides, detailed cutaneous examination revealed melanotic mucocutaneous macules in the lips and buccal mucosa [Figures and ]. As per the patient, one of his two younger brothers had similar lesions over the lips, but he was not available for examination. There was no history of hematochezia, melena, or weight loss among the siblings or any other family member.\nAfter 5 months of the first surgery, the patient was reoperated for taking down of ileostoma. During operation, colocolic intussusception was found in the transverse colon with a large mass palpable inside. This colocolic intussusception was resected with ileotransverse anastomosis. Postoperative period was uneventful, and the patient was discharged after 6 days. Histopathology of the mass showed expansion of the marginal zone of the lymphoid tissue along with sheets of heterogeneous population of small lymphocytes, suggestive of MALToma []. Immunohistochemistry showed CD20 positivity. Both proximal and distal margins were negative for malignant cells.\nAfter 1-year follow-up, the patient was asymptomatic. Colonoscopy revealed few pedunculated polyps in the rectum and sigmoid colon, but the surface was smooth. Yearly colonoscopy follow-up was planned to detect early malignant changes.
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.