A Rare Case of Persistent Multifocal Cribriform-Morular Thyroid Carcinoma

William Kuenstner; Po Zhao; Wen Lee; Carlos Garcia; Kenneth D Burman; Leila Shobab

doi:10.1016/j.aace.2024.01.002

A Rare Case of Persistent Multifocal Cribriform-Morular Thyroid Carcinoma

AACE Clin Case Rep. 2024 Jan 16;10(2):63-66. doi: 10.1016/j.aace.2024.01.002. eCollection 2024 Mar-Apr.

Authors

William Kuenstner¹, Po Zhao¹, Wen Lee², Carlos Garcia², Kenneth D Burman², Leila Shobab²

Affiliations

¹ MedStar Georgetown University Hospital, Washington, D.C.
² MedStar Washington Hospital Center, Washington, D.C.

Abstract

Background/objective: Cribriform-morular thyroid carcinoma (CMTC) was considered a variant of papillary thyroid carcinoma (PTC) but is a separate entity in the 2022 World Health Organization classification. CMTC has an association with familial adenomatous polyposis (FAP). Our objective is to report a case of CMTC who was subsequently diagnosed with FAP, to highlight these associated entities and implications for management.

Case report: A 15-year-old female with a history of iron-deficiency anemia and alpha-gal syndrome presented with several years of goiter and dysphagia. She also noted unintentional weight loss, abdominal pain, melena and hematochezia, and symptomatic anemia. Physical examination was significant for multiple thyroid nodules. Laboratory results revealed normal thyroid function and iron deficiency. Multiple nodules were visualized on thyroid ultrasound, and fine needle aspiration biopsy was consistent with PTC. Total thyroidectomy was performed with a revised diagnosis of multifocal CMTC, with administration of adjuvant radioactive iodine due to persistent disease. Genetic testing confirmed FAP and she was referred for upper endoscopy, colonoscopy, and an evaluation for colectomy.

Discussion: There are no best practice guidelines for management of CMTC. Management of CMTC is guided by FAP status; sporadic cases can be managed with hemithyroidectomy, while FAP-associated cases are better managed with total thyroidectomy. Recurrence is usually managed with surgical resection. The decision to treat with adjuvant radioactive iodine is often extrapolated from management of classic PTC.

Conclusion: Thyroid carcinoma in the setting of extensive family history of colorectal carcinoma should arouse suspicion for CMTC. Patients with CMTC should receive a referral for colonoscopy and genetic testing for FAP.

Keywords: cribriform-morular thyroid carcinoma; familial adenomatous polyposis; persistent cribriform-morular thyroid carcinoma; radioactive iodine ablation; thyroid carcinoma; β-catenin.

Publication types

Case Reports