mtc

A 16-year- old girl with history of hypertension was referred to the surgery department due to multiple hypertrophied lesions in both lips and on the lateral sides of the tongue. The lesions have been appeared gradually within the last year and only issued as a cosmetic concern. Which one could not be considered as the differential diagnosis of the patient? neurofibroma neurilemoma Leukoplakia Neuroma Correct answer: This figure demonstrates enlarged lip and hypertrophied tongue lesions which can be seen in neurofibroma, traumatic neuroma, neurilemoma, granular cell tumor, and neuroma; the net diagnosis is made by microscopic examination of a tissue biopsy. Leucoplakia as it is understood by its name is a white plaque with wide range of differential diagnosis; trauma, lichen planus, systemic lupus erythematous (SLE), leukoedema, and malignancy. On retrograde history taking, the patient had a total thyroidectomy due to medullary thyroid carcinoma (MTC) two years ago and right adrenalectomy due to pheochromocytoma last year. What would be the most probable diagnosis? Neurofibromatosis type 1 MEN 2A syndrome MEN 2B syndrome MEN 1 syndrome Correct answer: The classic combination of the mucosal neuroma, MTC, and pheochromocytoma together define the multiple endocrine neoplasia type 2B (MEN 2B). Patients with MEN 2B syndrome also might have marfanoid features. In Neurofibromatosis type 1, neurofibromas, multiple café- au- lait spots, iris hamartomas, skeletal abnormalities, glioma, and cognitive disorders are expected. MEN 2A is identical with MEN 2B but instead of marfanoid features and neuromas, parathyroid hyperplasia is seen. MEN 1 syndrome is characterized by pituitary adenoma, parathyroid hyperplasia, and pancreatic tumor. Due to the high fatality of MTC, early detection of MEN 2B cases and prophylactic thyroidectomy is of great importance and clinical suspicion to MEN 2B by non- endocrinologic features is critical in patients [1]. Currently, growing evidence is affecting our mindset about the clinical picture of MEN 2B syndrome. While MEN 2B patients are classically considered to be tall with marfanoid features, current studies report pediatric patients with proportionate short stature. It is also highlightable that intestinal ganglioneuromatosis associated with MEN 2B, should be considered in the differential diagnosis of Hirschsprung’s disease in children with constipation during early infancy.

Context: Inherited and sporadic medullary thyroid cancer (MTC) is an uncommon and medically challenging malignancy. Even if the extent of initial surgery is deemed adequate, the recurrence rate remains high, up to 50% in most series. Measurement of serum calcitonin is important in the follow-up of patients with MTC, and reliably reflects the existence of the disease.. Evidence Acquisition: There is no single sensitive diagnostic imaging method to reveal all MTC recurrences or metastases. Conventional morphologic imaging methods (U/S, CT, and MRI) and several methods of nuclear medicine have been used for this purpose with variable accuracy.. The main role of nuclear medicine imaging is the detection of residual or recurrent tumor in the postoperative follow-up. In this review we present the radiopharmaceuticals used in the diagnosis of MTC recurrence, and comparison among them.. The most used radiopharmaceuticals labelled with γ emitters are: Metaiodobenzylguanidine (MIBG), labelled with 131I or 123I, 111In-pentetreotide (Octreoscan), 99mTc-pentavalent dimercaptosuccinic acid (99mTc(V)-DMSA), and 99mTc-EDDA/HYNIC-Tyr3-Octreotide (Tektrotyd). The radiopharmaceuticals labelled with a positron-emitting radionuclide (β+), suitable for positron emission tomography (PET) imaging are: 18F-fluorodeoxyglucose (18F-FDG), 18F-fluorodihydroxyphenylalanine (18F-DOPA), and 68Ga-labelled somatostatin analogues (68Ga-DOTATATE or DOTATOC)..