Category: Other - Endocrine and Oncology, Calcium
Objective : Thyroid carcinoma comprises 1% of all malignancies, with differentiated thyroid cancer being the most common. Concomitant hematological malignancy is seen in 7% of cases. The association of sarcoidosis and lymphoproliferative disease, also known as sarcoidosis-lymphoma syndrome, is well-characterized but less commonly encountered.
Methods : Case
Results : Our patient is a 71 y/o woman with a left flank mass which was shown to be marginal zone B-cell lymphoma. She had cutaneous lesions in her neck, palate and parotid gland which improved with Rituxan. Surveillance FDG-PET-CT two years later revealed an FDG-avid l.2 cm left thyroid nodule with calcification, which turned out to be papillary thyroid carcinoma. She underwent total thyroidectomy and treatment with radioactive iodine 150 mCi and remained stable. Several years later, during a routine blood test, she was found to have elevated calcium levels to 15mg/dL. Simultaneously, she had also undergone neck ultrasound for thyroid cancer surveillance and found to have a left neck lesion which demonstrated abundant noncaseating granulomatous asteroid bodies. For further evaluation, she underwent repeat PET-CT which showed diffuse avidity in the mediastinum and hilar nodes and additional skin lesions consistent with lymphoma. Endobronchial ultrasound and biopsy of hilar nodes demonstrated nonnecrotizing granulomas consistent with sarcoid. Labs were also notable for elevated ACE level. Treatment was initiated with prednisone with note of improvement of lesions and calcium levels. Two years later, PETCT showed increased paratracheal and hilar lymphadenopathy and increase in the size and number of pulmonary nodules, and lesions in her upper and lower lip. The decision was made to restart weekly rituxan with resulting complete resolution of these lesions after four weeks.
The trio of these diseases has not yet been reported. It is also rare for the sarcoid to appear after, rather than before the lymphoproliferative disorder. The mechanism involves a dysfunction in the immunoregulatory pathways. A clone may escape in an environment with defective T suppressor cells regulation, thus resulting in the development of lymphoid malignancy. Alternatively, antineoplastic therapy in the treatment of the malignancy, could reduce suppressor T cells, leading to lymphocyte activation observed in sarcoidosis.
This case is unique in that it represents an undescribed constellation of two malignancies followed by sarcoidosis, with yet an incompletely understood mechanism. The presentation is also rather unusual in that her sarcoidosis was diagnosed as she presented with asymptomatic hypercalcemia, during follow up for her thyroid cancer.
Roselyn Mateo is a second year Clinical Fellow in Beth Israel Deaconess Medical Center in Boston, MA. She finished her Internal Medicine Residency in Rush University Medical Center and later on served as Chief Resident. Her clinical and research interests include Diabetes, Obesity, Lipids and Metabolism and Health Disparities.
Associate Professor Harvard Medical School
Beth Israel Deaconess Medical Center
Johanna Pallotta has practiced Endocrinology for over 50 years and her expertise in thyroid and parathyroid cancer is unparalleled. She is a master clinician and has been involved in the forefront of groundbreaking clinical practices in Endocrinology and advancement of the fellowship program.