Introduction to Nasal Chondromesenchymal Hamartoma

What is/are Chondromesenchymal Hamartoma?

Chondromesenchymal Hamartoma - Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumour of the nasal cavity predominantly described in infants. ^[1] Nasal Chondromesenchymal Hamartoma (NCMH) is a rare tumour of the sinonasal tract with a benign clinical behaviour. ^[2] Chondromesenchymal hamartoma (CMH) is a rare, benign lesion of the nasal cavity, paranasal sinuses, and skull base, composed of islands of hyaline cartilage in a myxoid background. ^[3] Nasal chondromesenchymal hamartoma (NCMH) is a rare benign lesion of the sinonasal tract in children and adolescent with orbital involvement. ^[4] Differential diagnoses include nasal-tip teratoma, nasal chondromesenchymal hamartoma (NCMH), and heterotopic nasal glioma. ^[5] Nasal chondromesenchymal hamartoma is discussed elsewhere in this issue. ^[6] However, the clinical phenotype of DICER1 pathogenic variation is not simply confined to a group of sarcomas arising in a number of extrapulmonary sites, but also non-sarcomas such as pituitary blastoma, pineoblastoma, ciliary body medulloepithelioma (CBME), chondromesenchymal hamartoma, multinodular goiter harboring papillary thyroid carcinoma, pediatric poorly differentiated thyroid carcinoma, multicystic hepatic lesions and cystic nephroma (CN). ^[7] Nasal chondromesenchymal hamartoma is a rare benign lesion of the sinonasal tract in children and adolescents. ^[8] DICER1 germline (associated with the inherited DICER1 syndrome) or somatic mutations have been linked to tumorigenesis in histogenetically diverse benign and malignant neoplasms in different organs including pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, nasal chondromesenchymal hamartoma, poorly differentiated thyroid carcinoma, thyroblastoma, intracranial sarcoma and gonadal Sertoli-Leydig cell tumors in addition to others. ^[9] ABSTRACT Nasal chondromesenchymal hamartoma is a rare benign tumor of the sinonasal tract in pediatrics and only few cases in infantile, early pediatric, and adolescent population have been reported. ^[10] The detection of the germline mutation in DICER1 provided the opportunity to identify and continue to recognize a number seemingly unrelated extrapulmonary neoplasms: Sertoli-Leydig cell tumor, gynandroblastoma, embryonal rhabdomyosarcomas of the cervix and other sites, multinodular goiter, differentiated and poorly differentiated thyroid carcinoma, cervical-thyroid teratoma, cystic nephroma-anaplastic sarcoma of kidney, nasal chondromesenchymal hamartoma, intestinal juvenile-like hamartomatous polyp, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma, primary central nervous system sarcoma, embryonal tumor with multilayered rosettes-like cerebellar tumor, PPB-like peritoneal sarcoma, DICER1-associated presacral malignant teratoid neoplasm and other non-neoplastic associations. ^[11] RESULTS We calculated the age at first neoplasm diagnosis (systematically ascertained cancers plus DICER1-associated neoplasms pleuropulmonary blastoma, cystic nephroma, and nasal chondromesenchymal hamartoma) in 102 female and male nonproband DICER1 carriers. ^[12] Nasal chondromesenchymal hamartoma (NCMH), a rare, benign, nasal cavity tumor, typically occurs in children. ^[13] kr Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal tract. ^[14] Histology revealed the diagnosis of chondromesenchymal hamartoma in ectopic thyroid tissue. ^[15] The hallmarks of DICER1 syndrome are certain rare tumors including pleuropulmonary blastoma; cystic nephroma; ovarian Sertoli–Leydig cell tumor; sarcomas of the cervix, kidneys and cerebrum; pituitary blastoma; ciliary body medulloepithelioma; and nasal chondromesenchymal hamartoma. ^[16] Emerging entities include human papillomavirus–related sinonasal carcinomas, SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1–deficient sinonasal carcinomas, renal cell-like adenocarcinomas, and chondromesenchymal hamartomas. ^[17] Other manifestations include ovarian Sertoli-Leydig cell tumor, cystic nephroma arising in childhood, multinodular goiter, thyroid carcinoma, anaplastic sarcoma of the kidney, embryonal rhabdomyosarcoma, and nasal chondromesenchymal hamartoma, in addition to other rare entities. ^[18]

rare benign lesion

Chondromesenchymal hamartoma (CMH) is a rare, benign lesion of the nasal cavity, paranasal sinuses, and skull base, composed of islands of hyaline cartilage in a myxoid background. ^[1] Nasal chondromesenchymal hamartoma (NCMH) is a rare benign lesion of the sinonasal tract in children and adolescent with orbital involvement. ^[2] Nasal chondromesenchymal hamartoma is a rare benign lesion of the sinonasal tract in children and adolescents. ^[3]

poorly differentiated thyroid

However, the clinical phenotype of DICER1 pathogenic variation is not simply confined to a group of sarcomas arising in a number of extrapulmonary sites, but also non-sarcomas such as pituitary blastoma, pineoblastoma, ciliary body medulloepithelioma (CBME), chondromesenchymal hamartoma, multinodular goiter harboring papillary thyroid carcinoma, pediatric poorly differentiated thyroid carcinoma, multicystic hepatic lesions and cystic nephroma (CN). ^[1] DICER1 germline (associated with the inherited DICER1 syndrome) or somatic mutations have been linked to tumorigenesis in histogenetically diverse benign and malignant neoplasms in different organs including pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, nasal chondromesenchymal hamartoma, poorly differentiated thyroid carcinoma, thyroblastoma, intracranial sarcoma and gonadal Sertoli-Leydig cell tumors in addition to others. ^[2] The detection of the germline mutation in DICER1 provided the opportunity to identify and continue to recognize a number seemingly unrelated extrapulmonary neoplasms: Sertoli-Leydig cell tumor, gynandroblastoma, embryonal rhabdomyosarcomas of the cervix and other sites, multinodular goiter, differentiated and poorly differentiated thyroid carcinoma, cervical-thyroid teratoma, cystic nephroma-anaplastic sarcoma of kidney, nasal chondromesenchymal hamartoma, intestinal juvenile-like hamartomatous polyp, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma, primary central nervous system sarcoma, embryonal tumor with multilayered rosettes-like cerebellar tumor, PPB-like peritoneal sarcoma, DICER1-associated presacral malignant teratoid neoplasm and other non-neoplastic associations. ^[3]

Nasal Chondromesenchymal Hamartoma

Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumour of the nasal cavity predominantly described in infants. ^[1] Nasal Chondromesenchymal Hamartoma (NCMH) is a rare tumour of the sinonasal tract with a benign clinical behaviour. ^[2] Nasal chondromesenchymal hamartoma (NCMH) is a rare benign lesion of the sinonasal tract in children and adolescent with orbital involvement. ^[3] Differential diagnoses include nasal-tip teratoma, nasal chondromesenchymal hamartoma (NCMH), and heterotopic nasal glioma. ^[4] Nasal chondromesenchymal hamartoma is discussed elsewhere in this issue. ^[5] Nasal chondromesenchymal hamartoma is a rare benign lesion of the sinonasal tract in children and adolescents. ^[6] DICER1 germline (associated with the inherited DICER1 syndrome) or somatic mutations have been linked to tumorigenesis in histogenetically diverse benign and malignant neoplasms in different organs including pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, nasal chondromesenchymal hamartoma, poorly differentiated thyroid carcinoma, thyroblastoma, intracranial sarcoma and gonadal Sertoli-Leydig cell tumors in addition to others. ^[7] ABSTRACT Nasal chondromesenchymal hamartoma is a rare benign tumor of the sinonasal tract in pediatrics and only few cases in infantile, early pediatric, and adolescent population have been reported. ^[8] The detection of the germline mutation in DICER1 provided the opportunity to identify and continue to recognize a number seemingly unrelated extrapulmonary neoplasms: Sertoli-Leydig cell tumor, gynandroblastoma, embryonal rhabdomyosarcomas of the cervix and other sites, multinodular goiter, differentiated and poorly differentiated thyroid carcinoma, cervical-thyroid teratoma, cystic nephroma-anaplastic sarcoma of kidney, nasal chondromesenchymal hamartoma, intestinal juvenile-like hamartomatous polyp, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma, primary central nervous system sarcoma, embryonal tumor with multilayered rosettes-like cerebellar tumor, PPB-like peritoneal sarcoma, DICER1-associated presacral malignant teratoid neoplasm and other non-neoplastic associations. ^[9] RESULTS We calculated the age at first neoplasm diagnosis (systematically ascertained cancers plus DICER1-associated neoplasms pleuropulmonary blastoma, cystic nephroma, and nasal chondromesenchymal hamartoma) in 102 female and male nonproband DICER1 carriers. ^[10] Nasal chondromesenchymal hamartoma (NCMH), a rare, benign, nasal cavity tumor, typically occurs in children. ^[11] kr Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal tract. ^[12] The hallmarks of DICER1 syndrome are certain rare tumors including pleuropulmonary blastoma; cystic nephroma; ovarian Sertoli–Leydig cell tumor; sarcomas of the cervix, kidneys and cerebrum; pituitary blastoma; ciliary body medulloepithelioma; and nasal chondromesenchymal hamartoma. ^[13] Other manifestations include ovarian Sertoli-Leydig cell tumor, cystic nephroma arising in childhood, multinodular goiter, thyroid carcinoma, anaplastic sarcoma of the kidney, embryonal rhabdomyosarcoma, and nasal chondromesenchymal hamartoma, in addition to other rare entities. ^[14]