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Other genetic parathyroid conditions
Published in Pallavi Iyer, Herbert Chen, Thyroid and Parathyroid Disorders in Children, 2020
MEN-1 is an autosomal dominant inherited (and rarely sporadic) syndrome with a high degree of penetrance that predisposes to tumors of the parathyroid gland, anterior pituitary gland, and entero-pancreatic endocrine cells (also remembered by the 3Ps: parathyroid, pituitary, and pancreas). These develop in 90%, 30–70%, and 30–40% of patients, respectively, by age 40. In addition these patient are at risk for thymic and bronchial neuroendocrine tumors, gastric enterochromaffin-like tumors, and adrenal tumors (5). First described by Dr. Paul Wermer in 1953, it is estimated to have a prevalence of 2–3/100,000. The MEN1 gene, discovered in 1997, is a tumor suppressor gene, located on the long arm of chromosome 11 (11q13), and codes for the 610 amino acid nuclear protein called Menin. With more than 1000 identified somatic and germline mutations, the exact mechanism by which mutation to this protein causes endocrine tumors remains unknown (6).
Breast
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Routine tests are usually not required; extensive work-up is rarely indicated, but in certain groups, selected investigations may be useful, e.g. prepubertal gynaecomastia (infants) – testicular examination/ultrasound may yield a significant number of functional endocrine tumours. Other clues include small testicles, decreased libido/fertility, lack of male hair or eunuchoid body.
Endocrine system
Published in Brian J Pollard, Gareth Kitchen, Handbook of Clinical Anaesthesia, 2017
Brian J Pollard, Gareth Kitchen
Assess for other endocrine tumours. Patients may be obese and hypertensive. Preoperative tumour localisation with CT, MR, ultrasound, angiography and selective venous hormone sampling is important to permit selective removal of the adenoma and avoid blind subtotal pancreatectomy. Palpation of the pancreas at surgery may produce sudden hormone release.
Multiple endocrine neoplasia type 1: a review of current diagnostic and treatment approaches
Published in Expert Opinion on Orphan Drugs, 2020
Francesca Marini, Francesca Giusti, Francesco Tonelli, Maria Luisa Brandi
MEN1-associated cutaneous benign tumors include collagenomas, facial angiofibromas, and lipomas (penetrance of 85%, 70% and 30%, respectively, by the age of 40), which commonly manifest as multiple, long-lasting, lesions. Their permanence helps differentiate them from temporary inflammatory skin lesions. MEN1 angiofibromas are commonly located in the upper lip and vermillion border of the lip. MEN1 collagenomas appear as skin-colored to slightly hypopigmented, round to oval papules of 0.2–2 cm in diameter, prevalently located on the upper part of the trunk and on the neck. These two types of skin manifestations are more common in MEN1 patients than in the general population (64% vs. 8% and 62% vs. 5%, respectively), and can be, therefore, used as clinical indicators of MEN1 syndrome [52]. Moreover, since they may develop prior to any clinical symptoms from endocrine tumors, they are also useful for the pre-symptomatic MEN1 diagnosis.
Postmenopausal endometriosis: drawing a clearer clinical picture
Published in Climacteric, 2018
Women with endometriosis have an increased risk of some other malignancies aside from ovarian cancer. A study of 20 686 Swedish women who were hospitalized with endometriosis during the period 1969–1983 showed an increased overall risk for cancer (SIR 1.2, 95% CI 1.1–1.3) during a mean follow-up of 11.4 years29. Significant increases were observed, not only for ovarian cancer (SIR 1.9, 95% CI 1.3–2.8), but also for breast cancer (SIR 1.3, 95% CI 1.1–1.4) and hematopoietic malignancies (SIR 1.4, 95% CI 1.0–1.8); this latter excess was largely driven by an excess risk of non-Hodgkin's lymphoma (SIR 1.8, 95% CI 1.2–2.6). In an extended follow-up study involving 64 492 women hospitalized for endometriosis from 1969 to 2000, the overall cancer risk became statistically insignificant (SIR 1.04, 95% CI 1.00–1.07)30. There were, however, continued elevated risks for ovarian cancer (SIR 1.43, 95% CI 1.19–1.71), endocrine tumors (SIR 1.36, 95% CI 1.15–1.61), non-Hodgkin's lymphoma (SIR 1.24, 95% CI 1.02–1.49) and brain tumors (SIR 1.22, 95% CI 1.04–1.41). These findings suggest that further attention be given to the risk of cancers other than ovarian cancer in women with endometriosis in their advanced ages.
Paraneoplastic Cushing syndrome, case-series and review of the literature
Published in Acta Clinica Belgica, 2018
Annelies Deldycke, Christel Haenebalcke, Youri Taes
There are two main factors that determine the prognosis in patients with an ACTH secreting tumor. The first prognostic factor is the histological type of the tumor. Patients with SCLC, thymic carcinoids, and medullary thyroid carcinoid have the worst prognosis. In patients with a SCLC, paraneoplastic Cushing syndrome is related to extended disease and is correlated with a very poor prognosis, worse than SCLC patients without Cushing syndrome. Patients with endocrine tumors have better prognosis. The second prognostic factor is the level of hypercortisolemia. The higher, the worse the prognosis. In patients with an occult tumor, the prognosis is generally better, provided that the hypercortisolemia is controlled [2,4–6,38–40].