title: Our experience of treatment of cribriform morular variant of papillary thyroid carcinoma; difference in clinicopathological features of FAP-associated and sporadic patients.
abstract: Cribriform-morular variant (CMV) is a comparably rare histological subtype of papillary thyroid carcinoma (PTC). This can be associated with familial adenomatous polyposis (FAP) due to APC gene mutations. In this study, we investigated the difference in the biological characteristics between FAP-associated and sporadic CMV. Between 1991 and 2010, 32 patients with CMV were treated in Kuma Hospital. Thirty-one of these underwent initial surgery for CMV in Kuma Hospital. Twelve patients were FAP-associated and the remaining 19 were sporadic CMV. All patients were female. Tumors of FAP-associated CMV were more frequently multiple than those of sporadic CMV. Patient age and tumor size did not differ between the two groups. Of 12 FAP-associated CMV, 5 were detected by thyroid nodule (thyroid precedent group) and 7 were detected by FAP (polyposis precedent group) as an initial manifestation. Patient age was younger and tumor size was smaller in the polyposis group than in the thyroid nodule group. All patients lacked extrathyroid extension on intraoperative finding and were node-negative on pathological examination. To date, two patients with FAP-associated CMV who initially underwent hemithyroidectomy (one in Kuma Hospital and one in another hospital) showed recurrence to the remnant thyroid during follow-up. None of the patients showed recurrence to other regions or died of carcinoma. Taken together, CMV is considered an indolent disease in our series. FAP-associated CMV showed multiple tumors more frequently than sporadic CMV. Total thyroidectomy is recommended for FAP-associated CMV, but extensive lymph node dissection is not necessary.
authors.affiliation: Department of Surgery, Kuma Hospital, Kobe, Japan. ito01@kuma-h.or.jp
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ito
authors.forename: Akira
authors.initials: A
authors.lastname: Miyauchi
authors.forename: Hideki
authors.initials: H
authors.lastname: Ishikawa
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Hirokawa
authors.forename: Takumi
authors.initials: T
authors.lastname: Kudo
authors.forename: Chisato
authors.initials: C
authors.lastname: Tomoda
authors.forename: Akihiro
authors.initials: A
authors.lastname: Miya
mesh_terms: D000230:Adenocarcinoma / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D000293:Adolescent; D000328:Adult; D002277:Carcinoma; D002291:Carcinoma, Papillary; D003937:Diagnosis, Differential; D005260:Female; D017491:Genes, APC / Q000502:physiology; D006801:Humans; D009362:Neoplasm Metastasis; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D016019:Survival Analysis; D000077273:Thyroid Cancer, Papillary; D013964:Thyroid Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D047368:Tumor Burden; D014463:Ultrasonography; D055815:Young Adult