Article

Malignant transformation of nasal chondromesenchymal hamartoma in adult: A case report and review of the literature

Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Histology and histopathology (Impact Factor: 2.1). 03/2013; 28(3):337-44.
Source: PubMed

ABSTRACT

Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumor arising in the sinonasal tract, predominantly involving infants and children. To date, only 27 cases are reported in the international literature and there have been no reported cases of malignant transformation. We present a 40-year-old female patient with nasal obstruction and bloody rhinorrhea. Computed tomography (CT) of the nose and paranasal sinuses confirmed a heterogeneous polypoid soft-tissue mass filling the nasal cavity and extending into the maxillary and ethmoid sinus. The patient underwent a complete radical resection. Histological and immunohistochemical analyses showed a portion of the mass was consistent with typical NCMH. However, some areas of mass exhibited cytological atypia, marked mitotic activity and foci of necrosis. The atypical mesenchymal spindle cells were immunoreactive for vimentin, CD99 and smooth muscle actin (SMA) diffusely. The cartilaginous cells were immunopositive for S-100 protein. Ki-67 index was high in atypical areas, accounting for 50%. A rapid mass recurrence was observed at the original site only 3 months after surgery. The final diagnosis of NCMH with malignant transformation was made. To our knowledge, this is the first report of malignant transformation occurring in an adult with NCMH. Although NCMH commonly develops in the neonate or young infants and exhibits benign histological appearance and favorable prognosis, there is a possibility of malignant transformation in adult patients. Thoroughly histological inspections are suggested to be necessary to accurately diagnose this tumor when it is encountered in adults.

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    • "Les hamartomes [84] [85] [86] [87] [88] [89] [90] sont des pseudotumeurs ; il s'agit de malformations congénitales caractérisées par la présence ectopique de tissus. Les hamartomes n'ont pas un pouvoir de prolifération tissulaire comme les tumeurs bénignes ou malignes mais leur développement semble s'autolimiter à partir d'un certain volume. "

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    ABSTRACT: I tumori benigni delle cavità nasali e sinusali hanno la particolarità di presentare un’estrema diversità anatomopatologica, mentre la presentazione clinica è spesso più monomorfa. Sono dominati in frequenza dal papilloma invertito. Clinicamente i tumori benigni nasosinusali possono manifestarsi con la presenza di segni rinologici o con la presenza di segni che testimoniano un’estensione del tumore alle ossa del volto, generando così una deformazione del volto, o con la presenza di segni di un’estensione extrasinusale (orbitale, meningea). I due esami diagnostici più utili sono la TC e la risonanza magnetica, che forniscono risultati diagnostici per caratterizzare il tumore e l’infiammazione che esso provoca e studiare con precisione le estensioni tumorali. La classificazione OMS dei tumori benigni nasosinusali comporta tre capitoli: i tumori ossei e cartilaginei, i tumori delle parti molli e i tumori epiteliali. Sono illustrate le caratteristiche di ogni tumore sul piano clinico, radiologico, istologico, evolutivo e terapeutico.
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