Case Presentation - Fall, 2009

Pigmented Medullary Carcinoma with Anaplastic Features

NOTE: The diagnosis and analysis for this case study were provided by an independent physician. All conclusions and opinions are those of the physician and not Hologic, Inc.

Clinical Data: 74 year old black female with a solid mass in the right mid to lower thyroid lobe.

Specimen type: FNA of thyroid

Case provided by: Theresa Piccin, CT(ASCP), Conway Medical Center, Conway, SC

Cytologic Diagnosis:
Melanin producing Medullary Thyroid Carcinoma with Anaplastic Features vs. Metastatic Malignant Melanoma

Biopsy Diagnosis:
After removal of right thyroid lobe, along with iron, chromogranin, Congo Red, calcitonin, thyroglobulin and HMB45 immunostains, the concluded diagnosis was: Pigmented Medullary Carcinoma with Anaplastic Features.

Hazard, et. al., described medullary carcinoma of the thyroid (MTC) as a separate entity in 1959. Calcitonin is produced by the tumor cells and confirms its origin from parafollicular C cells. Medullary carcinomas are known to produce an assortment of ectopic substances such as: serotonin, prostaglandin, adrenocorticotrophic hormone (ACTH), carcinoembryonic antigen (CEA), mucin, peptides and amines along with others. Melanin production in medullary carcinoma is extremely rare with only 10 cases reported in the literature.

The fine needle aspiration (FNA) yielded a small amount of thick, dark brown material. Microscopically the sample was cellular consisting of small cells with round nuclei to markedly enlarged, anaplastic cells with macronucleoli. Heavy pigmentation was present in the cytoplasm of the cells and in the background. Pigment was negative on iron staining.

Gross description:
Gross examination revealed a 65 gram portion of thyroid gland measuring 7.0 x 3.5 x 4.5 cm. Sectioning revealed an oval, dark red to black, pigmented mass measuring 5.0 cm. Portions of the mass appeared partially liquefied. The lesion appeared to be fairly well circumscribed without gross perforation of the gland. The tumor was described as an irregularly shaped portion of tan-yellow, fibroadipose tissue measuring 6.5 cm in greatest dimension. Multiple, small, rubbery nodules measured up to 0.8 cm were identified within the tissue.

The thyroid tumor showed sheets and nests of neoplastic cells. The periphery of the lesion appeared fairly well circumscribed. Diffusely throughout the tumor was a large amount of brown pigment within the cells. The neoplasm showed a mixture of round, polygonal and spindle cells as well as multinucleated cells. The nuclei of the neoplastic cells varied in size and shape with many being extremely pleomorphic and hyperchromatic. Scattered pigmented cells were identified within the vascular spaces. Also seen were deposits of an eosinophilic material within the tumor.

Prognosis and Treatment:
Due to the scarcity of reported cases, the prognosis is not exactly known. However, the overall survival of patients with MTC is around 86% at 5 years and 65% at 10 years. Treatment is typically a total thyroidectomy, unless there is evidence of distant metastases. External radiation and chemotherapy have been used as palliative care for recurrent tumors or metastasis. Radioactive iodine is not used in patients with MTC.

Our patient developed metastases to the left submandibular region and left lung approximately 4 years after first presentation. Her outcome is unknown.

Positive for calcitonin, chromogranin and HMB45
Negative for thyroglobulin, iron, Congo Red

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