A case of a 20 year old Japanese woman who developed thyroid cancer exhibiting unusual cribriform structures while being followed up for familial adenomatous polyposis/Gardner9s syndrome is reported. The patient presented with osteomas, pigmented retinal lesions, and adenomas of the duodenum and the papilla of Vater, in addition to numerous adenomatous polyps in the colorectum. On ultrasonography, the thyroid cancer was localised to the right lobe and was identified as an irregular, internal echo tumour with a peripheral hypoechoic zone, measuring 1.8 cm in diameter. Histological examination of the resected tumour showed a concomitance of papillary proliferation and cribriform structures with follicles of varying sizes. These features can be distinguished from sporadic thyroid cancer.
Figure 1 Patient 1: colonoscopy shows a flabby potbelly-shaped pedunculated polyp (A) with a reddish and uneven head (B). The elongated polyp is composed of edematous submucosa and normal colonic mucosa (arrows), containing a depressed form of tubular adenoma (arrowheads)
A rare case of primary jejunal malignant mixed tumor arising in a 49-64-old Japanese male with von Recklinghausen's disease is reported. The patient, who had a past history of partial gastrectomy due to duodenal ulcer, was admitted with a complaint of epigastric pain. Upper gastrointestinal examinations showed a huge polypoid tumor located in the efferent loop of the gastrojejunostomy site. Because the tumor was strongly suggestive of leiomyosarcoma on histological examination of biopsy specimens, laparotomy was performed. The resected tumor measuring 10 X 7 X 7 cm was composed of adenocarcinoma admixed with various sarcomatous components, including rhabdomyosarcoma, osteosarcoma, and other sarcomas. Immunohistochemical analysis also supported this diagnosis. The features of this tumor closely resembled malignant mixed mullerian tumor of heterologous type that develops in female genital organs. It is well known that patients with von Recklinghausen neurofibromatosis have an increased incidence of mesenchymal tumors and malignant neoplasias, and therefore, it seems that there is a possible relationship between the histogenesis of this peculiar tumor and the genetic abnormality in this patient.
Aim : The aim of this study was to classify the endosonographic features of gastrointestinal submucosal tumors. Methods : The endosonographic and clinicopathologic findings of 302 submucosal elevated lesions from 295 patients were compared. Results : Endoscopic ultrasound was able to clearly demonstrate the anatomical layer origin of the lesions. The internal echo patterns of the submucosal tumors could be categorized into seven types consisting of simple cystic (I), multicystic (II), solid cystic (III), hyperechoic (IV), isoechoic (V), hypoechoic (VI), and mixed solid (VII). Cysts, lymphangioma, and lipoma were characterized as type I, II, and IV images, respectively. Brunner’s gland hamartoma and heterotopic gastric mucosa showed various patterns of cystic and solid echoes (I–VII). Inflammatory fibroid polyps, granular cell tumors, and carcinoid tumors often manifested type VI images. Malignant tumors, particularly leiomyosarcoma, tended to show VII images. Conclusions : The endosonographic classification is considered to be important in the diagnosis of gastrointestinal submucosal elevated lesions.
