Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex
BACKGROUND: Carney complex (CNC) is an uncommon multisystem endocrine disorder with significant variability of clinical manifestations including mucocutaneous areas (pigmented lesions, myxomas, blue nevi, etc.), endocrine tumors (adrenal, pituitary, thyroid glands, or testicles), and non-endocrine t...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Vesnu Publications
2020-07-01
|
| Series: | ARYA Atherosclerosis |
| Subjects: | |
| Online Access: | https://arya.mui.ac.ir/article_10734_f684c8555da33e6a989ca17af1488feb.pdf |
| _version_ | 1811154599922368512 |
|---|---|
| author | Zivar Shirinpour Zahra Farhangiyan Nehzat Akiash Homeira Rashidi |
| author_facet | Zivar Shirinpour Zahra Farhangiyan Nehzat Akiash Homeira Rashidi |
| author_sort | Zivar Shirinpour |
| collection | DOAJ |
| description | BACKGROUND: Carney complex (CNC) is an uncommon multisystem endocrine disorder with significant variability of clinical manifestations including mucocutaneous areas (pigmented lesions, myxomas, blue nevi, etc.), endocrine tumors (adrenal, pituitary, thyroid glands, or testicles), and non-endocrine tumors [cardiac myxomas, psammomatous melanotic schwannomas (PMS), breast myxomas as well as ductal adenomas, and osteochondromyxomas]. To our knowledge, this is the second report of CNC in Iran, presenting with typical manifestations.Case Report: A 29-year-old man was referred to our clinic to evaluate the likelihood of CNC because of recurrent cardiac myxomas. He sometimes suffered from self-limited episodes of non-exertional palpitation, dyspnea, weakness, and pallor. He had some features of acromegaly (such as increase in acral size and frontal bossing). The laboratory tests revealed a high insulin-like growth factor 1 (IGF1) level, with no growth hormone (GH) suppression after oral glucose tolerance test (OGTT). Pituitary magnetic resonance imaging (MRI) showed a microadenoma (5.79 × 2.80 mm) of the pituitary gland; then, he was diagnosed with CNC, having the following major criteria: recurrent cardiac myxomas, skin myxomas, and acromegaly due to GH pituitary microadenoma, as well as minor criteria: multiple cafe´-au-lait (CAL) spots, several skin tags and moles, and thyroid nodules. In this patient, laboratory tests for Cushing’s syndrome were equivocal, whereas pheochromocytoma was proven biochemically but unexpectedly pathology did not confirm it. Rather, the pathology of the right adrenocortical specimen revealed nodular hyperplasia.CONCLUSION: For patients with recurrent cardiac myxoma, especially with skin myxoma, the diagnosis of CNC should be considered and the search for other associations should be done even in an asymptomatic patient. |
| first_indexed | 2024-04-10T04:18:25Z |
| format | Article |
| id | doaj.art-94a5311677b94298b887044277c4a080 |
| institution | Directory Open Access Journal |
| issn | 1735-3955 2251-6638 |
| language | English |
| last_indexed | 2024-04-10T04:18:25Z |
| publishDate | 2020-07-01 |
| publisher | Vesnu Publications |
| record_format | Article |
| series | ARYA Atherosclerosis |
| spelling | doaj.art-94a5311677b94298b887044277c4a0802023-03-11T12:58:31ZengVesnu PublicationsARYA Atherosclerosis1735-39552251-66382020-07-0116314615010.22122/arya.v16i3.208010734Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complexZivar Shirinpour0Zahra Farhangiyan1Nehzat Akiash2Homeira Rashidi3Assistant Professor, Diabetes Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, IranAssistant Professor, Diabetes Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, IranAssistant Professor, Atherosclerosis Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, IranAssociate Professor, Diabetes Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, IranBACKGROUND: Carney complex (CNC) is an uncommon multisystem endocrine disorder with significant variability of clinical manifestations including mucocutaneous areas (pigmented lesions, myxomas, blue nevi, etc.), endocrine tumors (adrenal, pituitary, thyroid glands, or testicles), and non-endocrine tumors [cardiac myxomas, psammomatous melanotic schwannomas (PMS), breast myxomas as well as ductal adenomas, and osteochondromyxomas]. To our knowledge, this is the second report of CNC in Iran, presenting with typical manifestations.Case Report: A 29-year-old man was referred to our clinic to evaluate the likelihood of CNC because of recurrent cardiac myxomas. He sometimes suffered from self-limited episodes of non-exertional palpitation, dyspnea, weakness, and pallor. He had some features of acromegaly (such as increase in acral size and frontal bossing). The laboratory tests revealed a high insulin-like growth factor 1 (IGF1) level, with no growth hormone (GH) suppression after oral glucose tolerance test (OGTT). Pituitary magnetic resonance imaging (MRI) showed a microadenoma (5.79 × 2.80 mm) of the pituitary gland; then, he was diagnosed with CNC, having the following major criteria: recurrent cardiac myxomas, skin myxomas, and acromegaly due to GH pituitary microadenoma, as well as minor criteria: multiple cafe´-au-lait (CAL) spots, several skin tags and moles, and thyroid nodules. In this patient, laboratory tests for Cushing’s syndrome were equivocal, whereas pheochromocytoma was proven biochemically but unexpectedly pathology did not confirm it. Rather, the pathology of the right adrenocortical specimen revealed nodular hyperplasia.CONCLUSION: For patients with recurrent cardiac myxoma, especially with skin myxoma, the diagnosis of CNC should be considered and the search for other associations should be done even in an asymptomatic patient.https://arya.mui.ac.ir/article_10734_f684c8555da33e6a989ca17af1488feb.pdfcarney complexacromegalymyxoma |
| spellingShingle | Zivar Shirinpour Zahra Farhangiyan Nehzat Akiash Homeira Rashidi Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex ARYA Atherosclerosis carney complex acromegaly myxoma |
| title | Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex |
| title_full | Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex |
| title_fullStr | Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex |
| title_full_unstemmed | Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex |
| title_short | Recurrent cardiac and skin myxomas along with acromegaly: A case report of carney complex |
| title_sort | recurrent cardiac and skin myxomas along with acromegaly a case report of carney complex |
| topic | carney complex acromegaly myxoma |
| url | https://arya.mui.ac.ir/article_10734_f684c8555da33e6a989ca17af1488feb.pdf |
| work_keys_str_mv | AT zivarshirinpour recurrentcardiacandskinmyxomasalongwithacromegalyacasereportofcarneycomplex AT zahrafarhangiyan recurrentcardiacandskinmyxomasalongwithacromegalyacasereportofcarneycomplex AT nehzatakiash recurrentcardiacandskinmyxomasalongwithacromegalyacasereportofcarneycomplex AT homeirarashidi recurrentcardiacandskinmyxomasalongwithacromegalyacasereportofcarneycomplex |