ABSTRACT
We report the case of a 24-year-old woman with familial adenomatous polyposis and diagnosed with cribriform-morular variant of papillary thyroid carcinoma. Neck ultrasound and computed tomography identified multiple nodules in the thyroid gland and neck lymph nodes. The cytological analysis was compatible with the diagnosis of papillary cancer of the thyroid. Total thyroidectomy with lymph node dissection was performed. The histological analysis established the diagnosis of cribriform-morular variant of papillary thyroid carcinoma. Despite preoperative findings suggesting an aggressive form of thyroid cancer with lymph node involvement, the final diagnosis was a variant of papillary thyroid carcinoma often associated with familial adenomatous polyposis and known to have a good prognosis.
Subject(s)
Carcinoma, Papillary/pathology , Multiple Endocrine Neoplasia/pathology , Thyroid Neoplasms/pathology , Antineoplastic Agents, Hormonal/therapeutic use , Carcinoma, Papillary/classification , Carcinoma, Papillary/diagnostic imaging , Carcinoma, Papillary/genetics , Carcinoma, Papillary/radiotherapy , Carcinoma, Papillary/secondary , Carcinoma, Papillary/surgery , Combined Modality Therapy , Estrogens , Female , Genes, APC , Goiter, Nodular/etiology , Goiter, Nodular/surgery , Humans , Iodine Radioisotopes/therapeutic use , Lymphadenitis/pathology , Lymphadenitis/surgery , Multiple Endocrine Neoplasia/genetics , Neck Dissection , Neoplasms, Hormone-Dependent/diagnostic imaging , Neoplasms, Hormone-Dependent/genetics , Neoplasms, Hormone-Dependent/pathology , Neoplasms, Hormone-Dependent/therapy , Progesterone , Prognosis , Radiography , Radiotherapy, Adjuvant , Thyroid Neoplasms/diagnostic imaging , Thyroid Neoplasms/genetics , Thyroid Neoplasms/radiotherapy , Thyroid Neoplasms/surgery , Thyroidectomy , Ultrasonography , Young AdultABSTRACT
Paragangliomas of the head and neck are uncommon neoplasms. They are usually benign, but tend to be locally invasive. Although surgical resection remains the definitive treatment, important issues about management arise when such lesions are inoperable. Beneficial effects of octreotide treatment have already been reported in a malign paraganglioma case. Here we report a 24 year old female with familial, bilateral, multiple paraganglioma in the head and neck region, who firstly presented with pulsatile tinnitus and hearing loss in her left ear. After embolization was performed, she underwent operation twice because of the gross tumor mass. No significant change in tumor size was determined after the operations, however there were no distant metastases. Although she experienced hypertension attacks, no hormonal overproduction was found in repeated measurements. As the tumor was unresectable, new alternative therapies were sought. Octreotide scintigraphy was positive in the tumoral tissue, so we began to treat her with somatostatin analogue octreotide. After a 16 month follow up period, an improvement of the performance status, the near normalisation of attacks and stabilization of tumor growth were achieved. However, in the last three visits, she began to experience symptoms more frequently and it had been necessary to increase the octreotide dose. She is now well and being followed up. In conclusion, the beneficial effects of octreotide treatment could be quantified by clinical, tumor and scintigraphic criteria. These data suggest that octreotide can be useful in the treatment of inoperable paragangliomas.