| Fact | Explanation |
|---|---|
| Lump at the base of the neck which moves with deglutition | The thyroid gland is attached to the larynx by means of the suspensory ligament of Berry which is a condensation of pretracheal fascia. Therefore the thyroid gland will move with swallowing. [1,2,3] |
| Hoarseness of voice | Due to malignant infiltration of the ipsilateral recurrent laryngeal nerve. [2] |
| Dysphagia | Due to malignant infiltration of esophagus. [1] |
| Respiratory difficulty | Due to malignant infiltration of trachea. [2] |
| Diarrhea | Due to increased intestinal electrolyte secretion secondary to high plasma Calcitonin levels. [1] |
| Weight loss | Due to loss of appetite because of liver metastasis. [2] |
| Bone pain | Due to bone metastasis causing lytic lesions. [1] |
| Palpitations, episodic sweating or any history of young hypertension | To detect concomitant pheochromocytoma which is a part of Multiple Endocrine Neoplasia Type II. [4] |
| Constipation, polyuria, polydipsia, memory problems, depresssion | These are symptoms of hypercalcaemia which is due to parathyroid hyperplasia which is a feature of MEN Type II. [4] |
| Family history of thyroid cancer, pheochromocytoma or sudden death. | MEN 2A, MEN 2B and FMTC (Familial Medullary Thyroid Carcinoma) are inherited as an autosomal dominant disorder. Occult pheochromocytoma can cause sudden unexplained death. [4] |
| Fact | Explanation |
|---|---|
| Dominant thyroid nodule at the base of the neck | This could be present in up to 74.2% of patients. [1,2,3] |
| Palpable cervical lymph nodes | Due to lymphadenopathy because of lymphatic spread. [3] |
| Jaundice | Due to liver metastasis. Acute liver failure also can happen. [1] |
| Bone tenderness | Due to bone metastasis. [2] |
| Elevated blood pressure | This is significant in a young patient as an indicator of pheochromocytoma.[4] |
| Tachycardia | Can indicate the presence of pheochromocytoma. [4] |
| Marfanoid habitus (high-arched palate, pectus excavatum, bilateral pes cavus, scoliosis) | To detect MEN Syndrome Type 2B. [4] |
| Neuromas on the eyelids, conjunctiva, nasal and laryngeal mucosa, tongue and lips. | These are features of MEN Syndrome Type 2B. [4] |
| Prominent hypertrophied lips. | Present in MEN Syndrome Type 2B. [4] |
| Multiple pruritic, hyperpigmented, lichenoid papules in tn the scapular area of the back. | Present in MEN Type 2A. [4] |
| Fact | Explanation |
|---|---|
| Metastases of gastroenteropancreatic (GEP) Neuro Endocrine Tumors (NET) | Presents with a similar clinical picture. Normal circulating Calcitonin levels and negative immunohistochemistry with Calcitonin antibodies rule out the diagnosis of Medullary Thyroid Carcinoma. [1] |
| Thyroid lymphoma | Presents with a thyroid nodule. Can be differentiated by Fine Needle aspiration Cytology. [2] |
| Follicular carcinoma of thyroid | This also presents with a solitary nodule. However it can't be differentiated from a follicular adenoma without histological evaluation. [2] |
| Papillary carcinoma of thyroid | Presents with a thyroid nodule. Can be differentiated by Fine Needle Aspiration Cytology (FNAC). [2] |
| Colloid nodules | Can present as a solitary thyroid nodule. Fine Needle Aspiration Cytology (FNAC) helps in diagnosis. [2] |
| Fact | Explanation |
|---|---|
| Fine Needle Aspiration Cytology | Can detect typical cytological features thus establishing the diagnosis. [1] |
| Serum Calcitonin levels | This is a highly sensitive test for early diagnosis of Medullary Thyroid Cancer but confirmatory stimulation testing with pentagastrin is necessary in most cases to identify true positive increases. [2,3] |
| Fact | Explanation |
|---|---|
| 24- hour urine analysis for catecholamine metabolites like VMA (Vanillylmandelic acid) and metanephrine. | In ruling out concomitant pheochromocytoma in patients with multiple endocrine neoplasia type 2A or 2B. Pheochromocytoma must be treated before Medullary Thyroid Carcinoma. [1,2] |
| Serum Calcium | To exclude hyperparathyroidism. [2] |
| Indirect layngoscopy | To assess vocal cord function prior to surgery. [3] |
| Fact | Explanation |
|---|---|
| Serum Calcitonin | To detect tumor recurrence. [1,2] |
| Serum carcinoembryonic antigen | To detect tumor recurrence. [1,2] |
| Fact | Explanation |
|---|---|
| Genetic testing to detect mutation of RET gene | Screening - All people with a family history consistent with MEN 2 (Multiple Endocrine Neoplasia) or FMTC (Familial Medullary Thyroid Carcinoma) should be screened with RET testing. For MEN 2B this should be done shortly after birth. For MEN 2A and FMTC this should be done before 5 years of age. [1,2] |
| Ultra Sound Scan of the neck to include the superior mediastinum, central and bilateral lateral neck compartments. | Staging - To detect lymph node metastasis. [2] |
| CT scanning of chest and neck | Staging - Only if there is regional lymph node involvement or calcitonin levels are more than 400pg/mL. To detect metastasis. [2] |
| Dual phase CT liver or MRI | Staging - To detect liver metastasis. [2,3] |
| Bone scintigraphy or MRI spine | Staging - To detect bone metastasis. [2] |
| Fact | Explanation |
|---|---|
| Radiotherapy | Palliative radiotherapy can play a valuable role in unresectable masses and painful bone metastases. [1] |
| Chemotherapy | This is now rarely used. Doxorubicin produces symptomatic response in <30% of cases; most are partial and of short duration. [1] |
| Palliative care | Gastrointestinal symptoms often respond well to symptomatic treatment (such as loperamide and/or codeine phosphate). Medical therapy should concentrate on symptom control. Somatostatin analogues are a possible alternative, which may decrease tumor peptide release. Symptomatic distant metastases may respond to surgery, EBRT, thermoablation and chemoembolization. Patients with known bony metastases may benefit from biphosphonates or denosumab. [1] |
| Fact | Explanation |
|---|---|
| Surgical management | To achieve loco-regional control (the neck and superior mediastinum), and in some patients biochemical as well as clinical cure. Patients with established MTC - total thyroidectomy and central compartment node dissection Patients with incidental, sporadic (RET negative), unifocal micro MTC <5 mm - completion thyroidectomy is not essential. Post-operative basal calcitonin should determine the need for further surgery. Patients with clinical or radiologically involved lymph nodes in the lateral compartment - selective lateral neck dissection of levels IIa–Vb. Ipsilateral prophylactic lateral neck dissection is recommended in the presence of central compartment node metastases. The need for lymph node dissection may not be apparent without histopathological confirmation of involved lymph nodes. Personalized decision Making is recommended based upon the probability of central compartment nodal metastases. [1,2,3] |
| Adjuvant therapy | External Beam Radio Therapy should be considered only once optimal surgery has been performed and if there is significant risk of local recurrence. Unlike differentiated thyroid cancer (DTC), radioiodine ablation or therapy are not options. [1,4] |