- Mesenchymal Hamartoma of the Liver in Beckwith-Wiedemann Syndrome: A Case Report.
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Seong Ho Yoo, Hyo Jin Park, Soo Yoen Cho, Chong Jai Kim
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Korean J Pathol. 2002;36(6):425-428.
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 Abstract
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- Beckwith-Wiedemann syndrome is a well-known overgrowth syndrome associated with the presence of a wide variety of anomalies and increased risk of cancers. Less frequently, benign neoplasms also develop. We report a female infant with Beckwith-Wiedemann syndrome who developed a mesenchymal hamartoma of the liver. The patient was born with macroglossia, ear lobe crease, and abdominal distension.
Laboratory data showed hypoglycemia, and magnetic resonance image revealed both adrenal enlargement, enhancing mass of the pancreas, and multiple hepatic nodules. The histologic findings of the resected distal pancreas and both adrenals were those of Beckwith-Wiedemann syndrome. Microscopic findings of the liver biopsy specimens were compatible with mesenchymal hamartoma. Hamartoma of the urinary bladder, cardiac fibrous hamartoma, and mixed hamartoma of the liver have been documented previously in association with Beckwith-Wiedemann syndrome. However, to our knowledge, this is the first case report of hepatic mesenchymal hamartoma in Beckwith-Wiedemann syndrome. Because of the paucity of hamartomas in childhood, we should be cautious of other features of Beckwith-Wiedemann syndrome and the present case extends the spectrum of tumor formation in this syndrome.
- Intestinal Metastasis of Osteosarcoma Presenting with Intussusception: A Case Report.
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Seong Ho Yoo, Min A Kim, Kyu Joo Park, Joon Koo Han, Sang Hoon Lee, Chong Jai Kim, Eui Keun Ham
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Korean J Pathol. 2002;36(4):271-273.
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Abstract
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- Metastatic osteosarcoma most commonly affects the lungs and other bones. Intestinal intussusception caused by metastatic osteosarcoma is very rare. We report a case of metastatic osteosarcoma of the intestine in a 39-year-old female. She underwent surgical resection of the left femur due to osteosarcoma and received additional chemotherapy 3 years ago. Pulmonary metastasis was found two years later and the patient complained of abdominal pain, nausea and vomiting after 8 months following excision of the lung nodules.
Abdominal computed tomography revealed intussusception with a suspected polypoid mass in the distal portion of the jejunum. The histologic findings of the resected bowel were those of osteosarcoma. This is the first case of documented intestinal metastasis of osteosarcoma in Korea. It is suggested that the tumor metastasis to the small intestine should be considered in patients with previous osteosarcoma, when the patient presents with acute abdominal symptoms and intussusception.
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