Paraganglioma presenting as a mesenteric cystic mass: A case report

Ilieşiu, Andreea; Ungureanu, Irena Antonia; Petca, Aida; Constantin, Maria Magdalena; Petca, R.; Şandru, Florica; Constantin, Traian; Dumitrașcu, Mihai Cristian

doi:10.3892/etm.2020.8805

Cited by 11 publications

(8 citation statements)

References 9 publications

(8 reference statements)

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“…CNC needs to be differentiated from Carney triad which is a very rare combination of unknown cause, associated with three different types of tumors: pulmonary chondroma, gastric leiomyosarcoma [very similar to gastrointestinal stromal tumor (GIST) presentation] and paraganglioma (69,70).…”

Section: Discussionmentioning

confidence: 99%

Dermatological and endocrine elements in Carney complex (Review)

et al. 2021

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Carney complex (CΝC) is a very rare, autosomal dominant, hereditary syndrome. Seventy percent of individuals with CNC have germline inactivating or deleting mutations of the CNC1 gene [currently known as protein kinase cAMP-dependent type I regulatory subunit α (PRKAR1A), located at the 17q22-24 chromosome level], with 30% of cases presenting with phosphodiesterase gene mutations. A member of the lentiginosis family, dermatological features include: skin pigmentation, cutaneous/mucosal myxomas, usually diagnosed by the age of 20 years (neonatal presentation is exceptional, requiring a meticulous differential diagnosis). Melanocyte-derived tumors such as epithelioid blue nevi (with different levels of pigmentation) and pigmented epithelioid melanocytoma (previously 'animal-type melanoma') are often found. Myxomas, mesenchymal tumors with mostly a benign pattern, may be recurrent. Primary cutaneous melanotic schwannoma are atypical, while non-skin sites are frequent. Corticotropinomas or somatotropinomas are part of the hereditary syndrome-related pituitary adenomas (representing 5% of all). Primary pigmented nodular adrenocortical disease involves bilateral cortical hyperplasia causing Cushing syndrome (CS) at an earlier age than non-CNC cases; osteoporotic fractures seem more prevalent compare to CS of other etiologies. Typically benign, a few cases of adrenocortical carcinoma have been identified. A total of 5% of familial non-medullary thyroid cancer is syndromic, also including CNC. CNC-related thyroid frame includes: hyperthyroidism, follicular hyperplasia/adenomas, follicular carcinoma (usually aggressive, bilateral or multifocal). Large cell calcifying Sertoli cell tumors of the testes have malignant behavior in adults; in children these may induce precocious puberty. Two particular mammary tumors are found: myxoid fibroadenomas and breast myxomatosis. Cutaneous/subcutaneous lesions, pigmented or not, or any focal swelling of non-identified cause needs careful examination, since dermatological elements are among the earliest and most discernable by which to detect lesions in CNC, a systemic condition with multi-level endocrine involvement.

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Section: Discussionmentioning

confidence: 99%

Dermatological and endocrine elements in Carney complex (Review)

et al. 2021

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“…Syndromic PHTP-related parathyroid tumors. PHTP is associated with a multitude of other non-parathyroid endocrine/neuroendocrine and non-endocrine tumors such as type IIA multiple endocrine neoplasia; but in cases with specific gene background as RET mutations, the parathyroid tumors actually underline a hyperplasia, not an adenoma (44)(45)(46)(47)(48). Since the relationship with genetic makeup and the presence of other tumors influence the timing of PHTP diagnosis and its frequency among a particular syndrome such as other less described neoplasia associations are still incompletely understood (for instance, meningioma and PTA), we consider this heterogeneous chapter a completely different topic and thus we did not discuss it here (47,48).…”

Section: Future Considerationsmentioning

confidence: 99%

Primary hyperparathyroidism-related giant parathyroid adenoma (Review)

et al. 2021

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Primary hyperparathyroidism (PHPT), an endocrine condition caused by a parathyroid adenoma (PTA) in 80-85% of the cases, has shifted in the modern era to a mildly symptomatic phenotype due to the prompt recognition of hypercalcemia and to a minimally invasive surgical approach which has a curative potential. Clinical complications of PHTH are either related to high calcium or parathyroid hormone [also parathormone (PTH)] or both, while the originating tumor typically is small, without local mass effects. A distinct entity is represented by giant PTA (GPTA) which is considered at a weight of more than 3 (3.5) grams. The present article is a review of the literature involving practical points of non-syndromic PHPT-related GPTA. Most authors agree that pre-operatory calcium and PTH are higher in GPTA vs. non-GPTA. However, the clinical presentation of PHPT may be less severe, probably due to local mass effects that bring the patient to an early medical evaluation. Age distribution, sex ratio, rate of successful pre-operatory location do not differ from non-giant PTA. Hypovitaminosis D is more frequent in PTA of higher dimensions. Post-operative hypocalcemia, but not recurrent/persistent PHPT, is expected, even hungry bone disease. A higher rate of atypia is described although the tumor is mostly benign. Unusual presentations such as cystic transformation, initial diagnosis during pregnancy or auto-infarction have been reported. The ectopic localization of PTA presented in almost 15% of all cases may also be found in GPTA. What are the exact cutoffs for defining GPTA is still an open issue. Contents 1. Introduction 2. Aim 3. Giant parathyroid adenoma: Concept around the size considerations 4. Pre-operative biological correlates 5. Relationship with vitamin D status 6. Histological issues 7. Ectopic PTA of large dimensions 8. Risk of post-operative hypocalcemia 9. Future considerations 10. Conclusions

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“…PHEO, respective PG, represent tumors with adrenal, respective extra-adrenal enterochromaphin cells which produce catecholamine (norepinephrine and epinephrine) and others amines like dopamine. These tumors are typically candidates to surgery in order to be cured, associating a high cardiovascular risk of complications before, during and after surgery (64,65). Neoplasia has a low incidence in the global population (2-8/1 million) but they represent up to 6 cases out of 1,000 hypertensive persons (66).…”

Section: Pheocromocytoma/paraganglioma and Skeletonmentioning

confidence: 99%

Neuroendocrine neoplasia and bone (Review)

et al. 2021

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This is a narrative review focusing on neuroendocrine neoplasia (NEN) and bone status, in terms of metastases and osteoporosis/fractures. One fifth of NEN have skeletal dissemination, this affinity being regulated by intrinsic tumor factors such as the C-X-C chemokine receptor 4 (CXCR4). Bone colonization impairs the patient quality of life, representing a surrogate of reduced survival. Patients with NEN without bone metastases may exhibit low bone mineral density, perhaps carcinoid-related osteoporosis, yet not a standardized cause of osteoporosis. Case-finding strategies to address bone health in NEN with a good prognosis are lacking. Contributors to fractures in NEN subjects may include: menopausal status and advanced age, different drugs, induced hypogonadism, malnutrition, malabsorption (due to intestinal resection, carcinoid syndrome), hypovitaminosis D, impaired glucose profile (due to excessive hormones such as glucagon, somatostatinoma or use of somatostatin analogues), various corticoid regimes, and high risk of fall due to sarcopenia. Pheocromocytoma/paraganglioma involve bone through malignant forms (bone is an elective site) and potential secondary osteoporosis due to excessive hormonal content and increased sympathetic activity which is a key player of bone microarchitecture/quality as reflected by low Trabecular Bone Score. Glucocorticoid osteoporosis is related to NEN-associated ectopic Cushing syndrome. Currently, there are a lack of studies to emphasis that excessive gut-derivate serotonin in NENs with carcinoid syndrome is a specific activator of bone loss thus a contributor to carcinoid-related osteoporosis.

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Paraganglioma presenting as a mesenteric cystic mass: A case report

Cited by 11 publications

References 9 publications

Dermatological and endocrine elements in Carney complex (Review)

Dermatological and endocrine elements in Carney complex (Review)

Primary hyperparathyroidism-related giant parathyroid adenoma (Review)

Neuroendocrine neoplasia and bone (Review)

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