Page 563 - Concise Pathology for Exam Preparation ( PDFDrive )
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548    SECTION II  Diseases of Organ Systems


                     •	 Widely  invasive  tumours  commonly  develop  metastasis,  and  about  50%  patients
                       succumb to their disease within 10 years.
                     •	 Minimally invasive follicular carcinoma has a 10-year survival greater than 90%.

                     Medullary	Carcinoma
                     •  Neuroendocrine neoplasm derived from the parafollicular or ‘C cells’.
                     Clinical features
                     •	 Secretes calcitonin, which has an important role in diagnosis and postoperative follow
                       up of patients.
                     •	 In addition, may secrete other polypeptide hormones, eg, somatostatin, serotonin and
                       vasoactive intestinal peptide (VIP).
                     •	 Sporadic  lesions  are  common  in  adults  (40–50  years);  cases  associated  with  MEN
                       syndrome are seen in younger patients/childhood.
                     •	 May present as/due to:
                       •	 A paraneoplastic syndrome, eg, diarrhoea due to excessive VIP or hypocalcaemia due
                         to increased serum calcitonin
                       •	 Mass symptoms
                     Gross morphology:
                     •	 Solitary/multiple lesions seen in both lobes of thyroid
                     •	 Bilateral and multicentric in a familial setting, and solitary and unilateral in a sporadic setting
                     •	 Firm, pale grey-tan and infiltrative
                     •	 Foci of haemorrhage and necrosis may be seen in larger lesions
                     Microscopy:
                     •	 Composed of polygonal to spindle-shaped cells, which may form nests, trabeculae and
                       follicles; rarely small, more anaplastic cells are the predominant cell type.
                     •	 Acellular amyloid deposits (derived from altered calcitonin) may be seen in the stroma.
                     •	 Multicentric  C-cell  hyperplasia  is  often  seen  in  the  surrounding  thyroid  in  familial
                       medullary carcinoma thyroid (absent in sporadic medullary carcinoma).
                     •	 Electron microscopy shows membrane-bound, electron-dense granules.
                     Prognosis: Prognosis of familial cancers is worse than sporadic (familial cancers tend to be
                       multiple and are associated with C-cell hyperplasia or micromedullary carcinomas ,1 cm).
                     Anaplastic	Carcinoma
                        It is an undifferentiated tumour derived from thyroid follicular epithelium.
                     Clinical features
                     •	 Presents as a rapidly enlarging bulky neck mass, which spread to contiguous structures
                     •	 Seen in older patients (mean age of 65 years)
                     •	 Fifty percent patients have a previous history of multinodular goitre
                     •	 Twenty percent have a previous history of a differentiated carcinoma
                     •	 Twenty  to  thirty  percent  have  a  concurrent  differentiated  thyroid  tumour  most
                       commonly PTC

                                        Genetic defects
                     Differentiated tumours            Anaplastic carcinoma
                                          Loss of P 53
                     Morphology
                     Highly anaplastic tumour, which may show any of the following histological patterns:
                     •	 Giant cell pattern (large pleomorphic giant cells)
                     •	 Spindle cell (sarcomatoid) pattern
                     •	 Mixed spindle cell and giant cell pattern
                     •	 Small cell pattern
                     Prognosis: Commonly metastasizes to lungs; is aggressive and fatal.


                     PARATHYROID GLAND
                     •	 Derived from developing pharyngeal pouches that also give rise to thymus
                     •	 Four glands (two each at upper and lower poles of thyroid)



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