ABSTRACT
Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic review of the literature on NCMH, we also report an unusual case of NCMH in an adolescent patient. A systematic review conducted following the PRISMA guidelines. PubMed, EMBASE and manual search through references of relevant publication were utilised to gather all published case-reports of NCMH. Data collected from each case-report for patient demographics, site and size of NCMH, clinical presentation, co-morbidities, diagnostic methods, treatment options and follow-up methods. The systemic review collected sixty-two case-reports of NCMH (including our case) affecting 42 men and 21 women (2:1 male to female ratio). Mean average age was 5.1 years (age range: 1 day to 70 years). The anatomical sites of the tumor were: nasal cavity (n = 17), paranasal sinuses (n = 30), orbital region (n = 17), and the base of the skull (n = 16). The reported clinical manifestations were nasal obstruction or congestion (n = 29), nasal mass (n = 27), epistaxis (n = 6), orbital symptoms (n = 14). NCMH is a very rare cause of nasal masses in infants and toddlers. Our case and previous case reports confirm that NCMH can mimic other benign and malignant tumors, therefore we should be vigilant for rare pathologies that lead to nasal masses. Recently the link between DIECR1 mutation with NCMH has been established, so NCMH should be considered in any patient with nasal or orbital symptoms with a history of DICER1-related tumor spectrum.
Subject(s)
Neoplasms , Female , Male , Humans , Adolescent , Child, Preschool , Infant , Ribonuclease III , DEAD-box RNA HelicasesABSTRACT
BACKGROUND: Primary mucinous eccrine carcinoma (PMEC) is a quite rare malignant tumor that typically involves the head and neck region in approximately 75% of patients and the periorbital area is the most common area. CASE PRESENTATION: A 57-year-old man was seen with a painless red mass involving the left lower eyelid for the past 7 months. Examination revealed a small mass measuring 0.6 cm with shiny red smooth appearance of the skin. H&E stained examination revealed a tumor mass that composed of polygonal cells in nests, lobules and islands separated by large amount of mucin. The neoplastic cells showed eosinophilic cytoplasm and round nuclei with mild pleomorphism. There was no necrosis, no atypical mitosis, no lymphovascular and perineural invasion. Rare mitotic figures were found. Tumoral nests present on all surgical margins. Primary MEC is a slow-growing neoplasm that may recur after incomplete surgical excision. This tumor is often clinically mistaken for other cutaneous tumors due to its variable appearance. Recurrent tumor tends to be locally invasive with a rare metastatic rate of 9.6%. CONCLUSION: As a result of the recurrence risk, patients should be followed up regularly. Thus, our patient was recommended to have a regular follow-up every six months.
ABSTRACT
Lipoma is not usual in the head and neck and the intrinsic form of isolated laryngeal lipoma is quite rare. Because isolated laryngeal lipoma symptoms are uncharacteristic and often has no systemic manifestation, clinical diagnosis is difficult. We describe an extremely rare case of intrinsic laryngeal lipoma.
