Long term outcome of chondromesenchymal hamartoma of sinonasal cavity: a rare entity.

Nasal chondromesenchymal hamartoma in the sinonasal cavity is an unusual entity mostly found in young infants and children. We present the case of a nasal chondromesenchymal hamartoma in a young female. NCMH has a favourable outcome that shows neither recurrence nor any malignant behaviour.

Transnasal endoscopic resection of nasal chondromesenchymal hamartoma in infancy: an analysis of 5 cases.

BACKGROUND: Nasal chondromesenchymal hamartomas (NCMHs) are extremely rare benign tumors that most commonly affect children in the first year of life. The purpose of this study was to investigate and summarize the characteristics of NCMH cases and the efficacy of transnasal endoscopic resection of NCMHs. METHODS: This is a retrospective study including 5 cases of infant diagnosed as NCMH between April 2016 and April 2020. Diagnostic techniques include nasoendoscopy, computerized tomography (CT) scan, magnetic resonance imaging (MRI) with contrast and microscopic and immunohistologic studies. Data collected included patient demographics, patient symptoms, radiographic findings, characteristics of tumor growth, follow-up time, recurrence, and postoperative complications. RESULTS: In 5 cases, 3 were males and 2 were females who aged 1, 2, 3, 6 months and 1 year, respectively. The size of the mass measured 1.6 cm*1.9 cm*1.8 cm at its smallest and largest was 4.0 cm*3.5 cm*3.0 cm. All five patients underwent tumor resection via transnasal endoscopic approach. Four tumors were completely removed, and one underwent partial resection, which was completely resected by midfacial degloving operation 13 months after the first surgery. There was no postoperative complication. The current postoperative follow-up period was 1 to 4 years, and no recurrence has been observed. CONCLUSIONS: Complete surgical resection of NCHM is necessary to resolve the symptoms and prevent recurrence. Transnasal endoscopic approach is a safe and effective choice for pediatric NCMH patients.

An Adult Case of Nasal Chondromesenchymal Hamartoma: Imaging Characteristics Including Diffusion-Weighted Images.

Nasal chondromesenchymal hamartoma (NCMH), a rare, benign, nasal cavity tumor, typically occurs in children. Differential diagnosis is difficult because NCMH often presents with non-specific findings, including cystic components and invasion of the surrounding area on T2-weighted magnetic resonance images. Here, we present a rare adult case of NCMH, with no clear hyperintensity on diffusion-weighted images (DWI), and bone remodeling on the tumor margins on computed tomography. To the best of our knowledge, this is the first report of DWI on NCMH, and these findings, which suggest benign disease, may be useful in diagnosing NCMH.

Chondro-osseous respiratory epithelial adenomatoid hamartoma of the nasal cavity.

Chondro-osseous respiratory epithelial adenomatoid hamartoma is a rare hamartomatous lesion of the nasal cavity, with only five cases reported in the literature to date. We report the case of a 3-year-old boy who presented with nasal obstruction and a mass in the left nasal cavity. The mass was completely resected on endoscopy. On microscopy, hamartomatous proliferation of respiratory-type glands admixed with islands of immature hyaline cartilage, characteristic of chondro-osseous respiratory epithelial adenomatoid hamartoma, was seen. Neither local recurrence nor distant metastasis was observed after 6 month follow up. Recognition of chondro-osseous respiratory epithelial adenomatoid hamartoma as a benign lesion is important to avoid unnecessary surgical procedures.

Nasal Glial Hamartoma: A New Type of Sinonasal Hamartomas.

Hamartomas are rare, tumour-forming, benign lesions that have been reported throughout the body that can resemble other malignant entities. Hamartoma subtypes can be distinguished based on their histological features. Sinonasal hamartomas may have presenting symptoms and radiological features that mimic other nasal neoplastic lesions. Therefore, it is essential to diagnose it accurately, as the treatment approaches can range from radical surgeries in malignant cases to a simple excision in hamartoma. In this paper, we report a novel case of sinonasal hamartoma, which demonstrates an unprecedented histological feature of glial tissue with astrocyte-like cells. Furthermore, we present the unconventional presenting symptoms and radiological features seen in this case that mimic the behaviours of nasal inverted papilloma (IP) lesions, thereby highlighting the need for careful investigation of such patients in order to distinguish both glial hamartoma and IP lesions. Concluding that identification of glial hamartoma as a new subtype of sinonasal hamartoma is crucial, as mistaking it for other lesions may subject patients to overly aggressive treatment and potential unnecessary harm.

Nasal Chondromesenchymal Hamartoma: A Rare Benign Lesion in Adult Female.

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign lesion of the sinonasal tract in children and adolescent with orbital involvement. NCMH is histologically composed of nodules of cartilage with cellular density variation and maturation of the chondrocytes, a myxoid to spindle cell stroma, focal osteoclast-like giant cells in the stroma, and erythrocyte-filled spaces. This lesion may present with a destructive pattern on imaging, highly suggestive of malignancy. Total endoscopic resection is the choice of treatment nowadays, however incomplete excision could result in tumor recurrence but rare.

Sinonasal Hamartomas: From Nasal Chondromesenchymal Hamartoma to Respiratory Epithelial Adenomatoid Hamartoma. Report of six Cases and Review of the Literature.

Sinonasal hamartomas are uncommon lesions of nasal and sinus cavities. Based on indigenous cellular components and characteristic histologic features, they are further classified into four entities: respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma (SH), chondro-osseous and respiratory epithelial hamartoma (CORE), and nasal chondromesenchymal hamartoma (NCH). REAH, SH, and CORE are seen in adult patients, while NCH predominantly occurs in newborns and infants. Morphologically REAH and SH are composed of respiratory epithelium and seromucinous glands, CORE is related to REAH but with additional feature of chondroid and/or osseous tissue, and NCH is composed of chondroid and stromal elements but devoid of epithelial component. All four lesions can present as sinonasal mass lesions and with associated obstructive symptoms. Given the rarity of these lesions, diagnosis can be challenging, especially in unusual clinical scenario. In this study, we report six cases of sinonasal hamartoma, including one case of NCH, one case of CORE, two cases of SH, and two cases of REAH. All cases were from adult patients including four men and two women. We also review the literature of the clinical and pathologic features of these rare lesions.

Nasal chondromesenchymal hamartomas in a cohort with pathogenic germline variation in DICER1.

BACKGROUND: Nasal chondromesenchymal hamartomas are benign, rare nasal tumors associated with DICER1 pathogenic germline variation. They can be locally destructive and recurrent if not completely resected. METHODOLOGY: In this single-center, case-control study, otorhinolaryngology evaluations and review of systems questionnaires of DICER1-carriers and controls enrolled in the DICER1 Natural History Study at the National Cancer Institute were collected. Review of these medical records were analyzed to determine if DICER1-carriers experienced different sinonasal clinical manifestations compared to controls. Additionally, the number of diagnoses of nasal chondromesenchymal hamartoma cases in the NCI DICER1 study was compared against the total person years of observation of DICER1-carriers in the study to determine the total number of cases per person-years of observation. Lastly, both the NCI DICER1 study and the International Pleuropulmonary Blastoma/DICER1 Registry were queried for unpublished cases of nasal chondromesenchymal hamartomas. RESULTS: There were no clinical differences in sinonasal symptomatology between DICER1-carriers and control patients seen in the ENT clinic. We observed of two cases of nasal chondromesenchymal hamartoma in a total of 555 person-years of monitoring DICER1-carriers. We include six unpublished nasal chondromesenchymal hamartoma cases. When combined with a comprehensive literature review, 38% of nasal chondromesenchymal hamartoma cases had at least one additional DICER1-associated tumor and 24% of the NCMH were found in the ethmoid sinus, the most commonly involved paranasal sinus. CONCLUSIONS: We quantify the risk of developing nasal chondromesenchymal hamartomas in our cohort of 236 DICER1-carriers, report six unpublished cases, and provide an updated review of the literature.

Nasal Chondromesenchymal Hamartoma with Skull Base and Orbital Involvement: Case Presentation.

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal tract in children with possible orbit and skull base involvement. We present the 57th published observation of this kind of tumor. A 25-month-old female patient presented with recurrent mass lesion of the sinonasal tract. According to her history, she had feeding difficulties and nasal obstruction since birth. She underwent partial resection at eight months of age via transfacial approach in the local hospital. Due to progression of tumor remnants, a second surgery was performed using an endoscopic endonasal approach resulting in subtotal resection. At 12 months of follow-up, a good postoperative result was observed with no signs of tumor progression despite incomplete resection. Histological and immunohistochemical examination of the biopsy specimens is presented. Comparison of specimens obtained from each of the two surgeries showed a difference in histological patterns. Endoscopic endonasal approach is the mainstay of surgical management. In case of incomplete resection, careful follow-up MRI studies should be recommended.

Endoscopic endonasal excision of nasal chondromesenchymal hamartoma with intracranial extension.

INTRODUCTION: Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign hamartoma of the sinonasal tract, predominantly involving infants and young children. METHODS: We report the case of a 3-year-old boy of NCMH with extension to anterior skull base. RESULTS: The tumor was completely resected piece by piece via an endonasal endoscopic approach. There is no recurrence 3 years after operation. CONCLUSIONS: We reported the case of NCMH extending to skull base was successfully resected by endonasal endoscopic approach.

Selected Diagnostically Challenging Pediatric Soft Tissue Tumors.

Many benign and malignant soft tissue tumors in children are challenging and their diagnosis requires knowledge of their vast diversity, histopathological complexity, and immunohistochemical, cytogenetic, and molecular characteristics. The importance of clinical and imaging features cannot be overstated. Soft tissue sarcomas account for 15% of all pediatric malignancies after leukemia/lymphoma, central nervous system tumors, neuroblastoma and Wilms tumor. This article discusses selected challenging pediatric soft tissue tumors with an update on recently described entities.

DICER1-pleuropulmonary blastoma familial tumor predisposition syndrome: a unique constellation of neoplastic conditions.

Germline mutations in DICER1 are associated with increased risk for a wide variety of neoplastic conditions, including pleuropulmonary blastoma (PPB), cystic nephroma, nasal chondromesenchymal hamartoma, ovarian Sertoli-Leydig cell tumors, botryoid embryonal rhabdomyosarcoma of the uterine cervix, ciliary body medulloepithelioma, pineoblastoma, pituitary blastoma and nodular thyroid hyperplasia or thyroid carcinoma. These tumors may be seen in isolation or in constellation with other characteristic tumor types in individuals or family members. Here we describe the medical history of a child with a heterozygous, loss of function germline DICER1 mutation and multiple tumors associated with the syndrome.. Although germline mutations in DICER1 are rare, tumors of these types will be seen by practicing pathologists and should prompt consideration of an underlying DICER1 mutation.