Case Keport PARATHY Rold CARCINOMA OCCURRING IN
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Case Keport PARATHY ROlD CARCINOMA OCCURRING IN MULTIPLE ENDOCRINE NEOPLASIA TYPE I Kurt S. Kodrofi M.D. EA.C.E.,l Claude Lieber, M.D., EA.C.S.,2 and Lester D. R. Thompson, M.D3 ABSTRACT INTRODUCTION Objective: To describe a patient with multiple The multiple endocrine neoplasia type I (MEN-I) syn- endocrine neoplasia type I (MEN-I) in whom parathyroid drome is characterized by neoplasms of the parathyroid, carcinoma developed. anterior pituitary, and pancreatic islet cells (1,2) as well as Methods: A case report is presented, including tumors in several other tissues, such as bronchial, thymic, detailed history, laboratory findings, and pathologic and gastric carcinoids, adrenocortical tumors, lipomas, features. and, possibly, thyroid tumors (3). Hyperparathyroidism is Results: A 49-year-old man with a known history of the most common feature to manifest in patients with this MEN-I syndrome had hypercalcemia and profoundly disorder, reaching a prevalence of almost 100% by age 40 increased parathyroid hormone levels. Seven years earlier, years; it is usually due to chief cell hyperplasia or adeno- he had been diagnosed with a gastrinoma, prolactinoma, ma (1). The recommended initial treatment for this cause and primary hyperparathyroidism. A neck exploration at of primary hyperparathyroidism is surgical intewention- that time consisted of resection of a right inferior parathy- a subtotal parathyroidectomy or total parathyroidectomy roid gland and parathyroid tissue in the thyroid gland as in conjunction with autotransplantation, primarily because well as biopsies of two left-sided glands, after which of the high rate of recurrences with lesser procedures microscopic examination revealed parathyroid hyperpla- (4,5). Recurrences usually result from continued growth sia and his serum calcium levels normalized. On reexplo- of residual hyperplastic tissue in patients who undergo less ration of the neck, resection revealed pathologic f~ndings than total parathyroidectomy (4,5). consistent with parathyroid carcinoma. Herein we describe a patient with MEN-I who had Conclusion: Parathyroid carcinoma has rarely been documented gastrinoma, prolactinoma, and hyperparathy- reported in the setting of MEN-I. This case illustrates the roidism, in whom hypercalcemia recurred because of a need for near-complete resection of parathyroid tissue in parathyroid carcinoma. The carcinoma was discovered in the patient with MEN-I. Subtotal or total parathyroidecto- the neck of the patient, who had previously been operated my in conjunction with forearm autotransplantation on for benign parathyroid hyperplasia. should be performed, not simply to prevent recurrence of benign disease but also to prevent the potential develop- CASE REPORT ment of carcinoma over time. (Endocr Pract. 1999; 5347-349) A 49-year-old man came to the Wilrnington Veterans Affairs ~&icalCenter because of nephGlithiasis. His serum calcium concentration was 12.8 mg/dL. The patient had been well until he was 34 years old, when he had passed a kidney stone. At age 43 years, he had a recurrence of kidney stones and was found to have a serum calcium concentration of 13.3 mn/dL and an increased intact parathyroid hormone (PTH) level (310 Submitted for publication October 7,1998 pg/mL). During further evaluation, he was found to have Accepted for publication March 3,1999 a fasting serum gastrin of 1,196 pg/mL (normal, 400) and From the l~e~artmentof Medicine and 2~epartmentof Surgery, Wilmington Veterans Affairs Medical Center, Wilmington, Delaware, and a serum gastrin after secretin stimulation of 2,005 pg/mL. 3~epartmentof Endocrine and Otorhinolaryngologic-Head & Neck Magnetic resonance imaging of his pituitary gland Pathology, Armed Forces Institute of Pathology, Washington, DC. revealed an 8-rnm sellar mass. A serum prolactin level at The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of that time was 76 ng/mL (normal, 45). the US Department of the Navy or the Department of Defense. Surgical exploration of the patient's neck revealed an Address correspondence and reprint requests to Dr. K. S. Kodroff, Division enlarged right inferior parathyroid gland (1.0 by 0.5 cm). of Endocrinology, Bronx-Lebanon Hospital Center, 1650 Grand Concourse The right superior parathyroid gland was not found despite Avenue, Bronx, NY 10457. 0 1999 AACE. a thorough search. A palpated nodule in the right upper ENDOCRINE PRACTlCE Vol. 5 No. 6 NovemberIDecember 1999 347 348 MEN Type I and Parathyroid Carcinoma, Kodroff et al. lobe of the thyroid gland was excised (and later deter- (normal, 1.5 to 9.3), follicle-stimulating hormone 4.7 mined to be parathyroid tissue by histologic examination). mIU/mL (normal, 1.4 to 18.I), vasoactive intestinal pep- Exploration of the left side of the neck revealed normal- tide <lo pg/mL (normal, 23 to 63), and 24-hour urinary sized superior and inferior parathyroid glands. Biopsy free cortisol 34 pg/24 h (normal, <105). specimens of these glands were obtained, and the explo- Radiographic studies of the neck and sella turcica ration was terminated. were performed. Magnetic resonance imaging disclosed a Macroscopic examination of the surgical specimens pituitary mass in the sella turcica that indented the inferi- revealed an aggregate weight of 900 mg for the parathy- or aspect of the optic chiasm. A sestamibi parathyroid scan roid tissue removed (right inferior = 750 mg, right upper revealed avid uptake in the right side of the neck. pole of thyroid = 94 mg, left superior = 38 mg, and left Treatment with bromocriptine was begun, and a proton inferior = 18 mg). Histologic examination demonstrated pump inhibitor was used. an increased cellularity of the parenchymal cells in con- On admission to the hospital for the planned neck junction with a decrease in the amount of adipose tissue. exploration, the patient complained of profound fatigue. The cells were arranged in vague nodules and cords. The The serum calcium level was 15.5 mg/dL. He was hydrat- dominant cell type was chief cell, although several small ed with saline and given an intravenous infusion of 45 mg oncocytic nodules were noted. The oncocytic cells con- of pamidronate. One week later, and 7 years after the ini- tained abundant eosinophilic cytoptasm surrounding tial operation, reexploration of the neck was performed. A hyperchromatic nuclei without nucleoli. The ratio of thorough search of the right side of the neck initially nucleus to cytoplasm was decreased because of an revealed no parathyroid tissue; however, within the increase in the amount of cytoplasm. No residual unin- excised right lobe of the thyroid gland, a mass (3.5 by 3.0 volved or atrophic parathyroid tissue was identified, nor by 3.0 cm) was found that proved to be parathyroid tissue were mitotic figures, necrosis, hemorrhage, and fibrosis on frozen section. seen. Moreover, no adherence to the thyroid gland was Histologically, tumor cells were identified in immedi- noted. Collectively, these features were diagnostic of ate association with the thyroid parenchyma, surrounded parathyroid hyperplasia. and intersected by dense bands of acellular, fibrous con- Postoperatively, the serum calcium level was 9.7 nective tissue. The tumor cells were seen in the surround- mgIdL, and there was no record of postoperative hypocal- ing adipose connective tissue. At the edge of the main cemia. The patient was dismissed from the hospital. With mass, vascular invasion of small to medium-sized vessels the exception of subsequent treatment with a proton pump could be identified just beyond the thickened capsule. inhibitor for gastrinoma, he was lost to endocrine follow- Although capsular transgression may have been present, UP. distortion at the periphery and areas of dense fibrous con- During the 7 years before the current consultation, the nective tissue scarring made this determination impossi- patient had had intermittent diarrhea and weight gain (36.3 ble. The tumor cells were arranged in trabeculae, with kg). He denied having headache, erectile dysfunction, areas of spindling. An overall monotonous pattern of the change in shoe size, hypertension, or hoarseness. He had cells showed an increased ratio of nucleus to cytoplasm, no family history of MEN-I or of hyperparathyroidism, condensed nuclear chromatin, and prominent nucleoli. and he had never had irradiation to his neck. His only Mitotic figures were noted along with areas of individual medication was omeprazole, 30 mg daily. On examina- cell necrosis (apoptosis). The pathologic features were tion, he was in mild distress attributable to flank pain. His consistent with a diagnosis of parathyroid carcinoma (6,7) blood pressure was high (16011 10 mm Hg), but the rest of (Fig. 1 and 2). his vital signs were within normal limits. He was obese but Two days postoperatively, the intact PTH level was did not have the characteristic appearance of a patient with 47 pg/mL. Two weeks postoperatively, the serum calcium Cushing's syndrome or acromegaly. Visual fields were level declined to 9.7 mg/dL in association with a serum intact to confrontation. The thyroid gland was difficult to albumin concentration of 3.5 g/dL and an intact PTH level palpate because of obesity. No neck masses were felt. The of 60.7 pg/mL. At 12 weeks postoperatively, the PTH abdomen was soft; no abdominal masses and no level was 61.2 pg/mL, the serum calcium concentration hepatomegaly were detected. The testicles were 5 cm in was 10.1 mgIdL, and the serum albumin was 3.7 g/dL. At length bilaterally and of normal consistency. Gyneco- 20 weeks, the intact PTH was 68.4 pgImL, and the serum mastia, striae, peripheral edema, and rashes were not calcium was 10.5 mg/dL. present. Laboratory studies revealed the following: semm cal- DISCUSSION cium 12.3 mg/dL (normal, 8.4 to 1~0.2),phosphorus 2.8 mg/dL (normal, 2.5 to 4.9, intact PTH (imrnunoradiomet- Parathyroid carcinoma in the setting of MEN-I is ric assay) 243 pg1mL (normal, 12 to 72), plasma glucose extremely rare.