Prevalence of C‐cell hyperplasia and medullary thyroid carcinoma in a consecutive series of pheochromocytoma patients

Jansson, Svante; Hansson, Göran; Salander, Håkan; Stenström, Gunnar; Tisell, Lars‐Erik

doi:10.1007/bf01654922

Cited by 14 publications

(6 citation statements)

References 19 publications

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“…The prevalence of lymph node metastases at diagnosis has varied between 25% and 62% in several other studies. [ 18 19 ] Metastasis from MTC occurs via lympho-vascular pathways, spreading to lymph nodes of neck and mediastinum and then further onwards to the lung, liver and bones. [ 20 ] Some patients with advanced MTC can develop obstructive symptoms, e.g., dysphagia, due to local growth, while those with distant metastasis can have flushing and diarrhoea due to high levels of calcitonin.…”

Section: Discussionmentioning

confidence: 99%

Medullary Thyroid Cancer: An Experience from a Tertiary Care Hospital of a Developing Country

Wang

Khan

Esbhani

et al. 2022

Indian Journal of Endocrinology and Metabolism

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Background: Medullary thyroid carcinoma (MTC) is a rare type of thyroid cancer that occasionally occurs as part of MEN2A. The universal treatment of MTC is total thyroidectomy with central lymph node dissection. For disease progression, carcinoembryonic antigen (CEA) and calcitonin (CTN) need to be followed. Our aim was to study the presence and patterns of the above-mentioned characteristics of MTC in our population. Methodology: This retrospective study was conducted in a tertiary care hospital of Pakistan in which data of thirty-two medullary thyroid cancer patients over the past 20 years were reviewed and analysed after fulfilment of inclusion criteria. Their clinical, pathological, biochemical and treatment modalities were recorded through a retrospective review of their medical record files. Results: The mean age of patients was 42.88 ± 2.67 years in our study, with a male-to-female ratio of 2:1. Patients with sporadic MTC were 68.8%, while 31.2% were familial. The rates of metastasis were highest in bones followed by lungs and liver. Total thyroidectomy was performed in 26 (81.2%) patients and among those chemotherapy and XRT were performed in one and two patients, respectively. Histologically, the mean tumour size was 7.62 ± 3.64 cm. Median pre-surgery calcitonin was 5756 pg/ml that decreased to 29.3 pg/ml post-surgery. Median pre-surgery CEA level was 246.5 ng/ml that decreased to 6.39 ng/ml post-surgery. Two patients were RET positive. Conclusion: MTC usually presents in the fourth decade of life with male predominance and mostly sporadic occurrence. Total thyroidectomy with subsequent serial calcitonin and CEA levels thereafter are the mainstay of treatment and follow-up.

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Section: Discussionmentioning

confidence: 99%

Medullary Thyroid Cancer: An Experience from a Tertiary Care Hospital of a Developing Country

Wang

Khan

Esbhani

et al. 2022

Indian Journal of Endocrinology and Metabolism

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“…In 1982, the prevalence of C-cell disease of the thyroid was investigated in 56 patients by serum calcitonin determinations after provocation with pentagastrin. Two patients refused to participate in this investigation and 6 had died [15]. The 16 patients operated on since 1982 were tested for hypercalcitoninemia before they left the hospital after adrenal surgery.…”

Section: Methodsmentioning

confidence: 99%

Multiple neuroectodermal abnormalities in pheochromocytoma patients

et al. 1988

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A series of 80 consecutive pheochromocytoma patients operated on from 1956 to 1985 were investigated for other neuroectodermal abnormalities. Such abnormalities were found in 23 (29%) patients. Medullary thyroid carcinoma was the most common associated neuroectodermal abnormality followed by von Recklinghausen's neurofibromatosis. Other abnormalities were intracranial tumors, midgut carcinoid, and parathyroid hyperplasia. All 6 patients with multiple pheochromocytomas had other neuroectodermal abnormalities. In the total series, the 14 patients with hereditary disease had multiple neuroectodermal abnormalities (MNA). Hypertension occurred significantly less often in MNA patients (p<0.05). All the MNA patients had benign adrenal pheochromocytoma. In 7 of 9 normotensive patients, pheochromocytoma was suspected because of the presence of other neuroectodermal abnormalities. None of these 9 patients had any cardio‐ or cerebrovascular complications, but these occurred in 22% of the hypertensive patients. No patient in the series died from pheochromocytoma, but 4 of 5 deceased MNA patients died as a result of their associated neuroectodermal disease. During the last decade, 40% of the pheochromocytoma patients had MNA diagnosed compared to 20% during the previous period. An increased observation for other neuroectodermal abnormalities in pheochromocytoma patients and for pheochromocytoma in patients with neuroectodermal abnormalities should lead to earlier diagnosis and a better prognosis for the patients concerned.

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“…In the familial forms there are associated endocrine or neuroendocrine lesions (Table I) Sipple syndrome (MEN type II or IIA) also known as "Classical MEN2A" consists of medullary thyroid cancer and C-cell hyperplasia, adrenal pheochromocytoma and adrenal medullary hyperplasia, and parathyroid hyperplasia (52,53). Although most affected patients will have the complete syndrome, not every patient will manifest each of these lesions.…”

Section: Clinical Presentation and Geneticsmentioning

confidence: 99%

C-cells and their associated lesions and conditions: a pathologists perspective

Baloch¹,

LiVolsi²

2015

TJPATH

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This paper updates the histopathology and cytopathology of thyroid tumors and proliferations derived from the para-follicular or C cells. Beginning with an historical over view, including the recognition of medullary thyroid carcinoma as a distinct histologic entity, its relationship to the hormone, calcitonin, (which was discovered in the same decade) and to thyroid C cells, medullary carcinoma and its variants are reviewed. The molecular biology of the tumors and the associated mutations in the tumors (somatic mutations) are discussed. Additionally the genetic features (germline mutations) including familial clusters and associations with other endocrine and neuroendocrine lesions are reviewed. Screening for the tumor and its precursors is included with a review of the latest American Thyroid Association guidelines for treatment as well as timing and approach to surgery. Tabular data of specific germline mutations and their relationships to tumor virulence, and prognosis are illustrated. Precursor and early C cell lesions such as C-cell hyperplasia and micro-medullary carcinoma are discussed. Difficulties and controversies in the definition of C-cell proliferations which are neoplastic and those which are "reactive" are reviewed. The entity of medullary microcarcinoma or medullary microcarcinoma is illustrated and the distinction between C cell nodules and microcarcinoma is defined using the latest available criteria. Finally the latest approved chemotherapeutic agents and their results in metastatic medullary thyroid carcinoma are included.

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Prevalence of C‐cell hyperplasia and medullary thyroid carcinoma in a consecutive series of pheochromocytoma patients

Cited by 14 publications

References 19 publications

Medullary Thyroid Cancer: An Experience from a Tertiary Care Hospital of a Developing Country

Medullary Thyroid Cancer: An Experience from a Tertiary Care Hospital of a Developing Country

Multiple neuroectodermal abnormalities in pheochromocytoma patients

C-cells and their associated lesions and conditions: a pathologists perspective

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