Abstract:A mesenchymal chondrosarcoma of the nasal region in a neonate is described. Problems of histological interpretation and management are discussed.
“… 2,5–7,23,24,26 Because of the inherent technical difficulties in completely removing tumors from the sinonasal tract, three‐dimensional CT scanning may also improve planning of composite resections, 27 which were performed in five of the patients in the present series and those reported in the literature (Tables I and II). 12,13,15 …”
Section: Discussionmentioning
confidence: 99%
“…Within the sinonasal tract, the maxillary sinus (n = 11) is most commonly affected, followed by the ethmoid sinuses (n = 8) and the nasal cavity (n = 6) (Table III). 11–17 …”
Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
“… 2,5–7,23,24,26 Because of the inherent technical difficulties in completely removing tumors from the sinonasal tract, three‐dimensional CT scanning may also improve planning of composite resections, 27 which were performed in five of the patients in the present series and those reported in the literature (Tables I and II). 12,13,15 …”
Section: Discussionmentioning
confidence: 99%
“…Within the sinonasal tract, the maxillary sinus (n = 11) is most commonly affected, followed by the ethmoid sinuses (n = 8) and the nasal cavity (n = 6) (Table III). 11–17 …”
Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
“…[3][4][5] In the literature, NCMHs are invariably diagnosed in infants or in early childhood. [1,2,[6][7][8][9][10][11][12][13][14][15] Patients with NCMHs commonly present with an intranasal mass that causes symptoms such as nasal obstruction, rhinorrhea and epistaxis. The symptoms and clinical presentation of the patient are mostly determined by the size and location of tumor.…”
Section: Discussionmentioning
confidence: 99%
“…Proptosis, enophthalmos or impairment of eye movement can be the presenting symptoms or findings in cases with orbital involvement. [2,6,[9][10][11][12][13][14][16][17][18][19] Intracranial extension of the tumor can result in neurologic manifestations. [2,17] Symptoms or signs such as difficulties in respiration or nourishment, epistaxis, rhinorrhea, middle ear effusion can be encountered due to tumor size and site.…”
Nasal chondromesenchymal hamartoma (NCMH) is a rare nasal benign tumor, which arises from the nasal cavity or paranasal sinuses. In this article, we present a five-year-old male patient with rhabdomyosarcoma unity in remission that emerged with nasal obstruction. Synchronous diagnosis of pediatric tumors such as pleuropulmonary blastoma in the literature is a remarkable finding. We found a mass within the left nasal cavity originating from superior portion of nasal septum, extending to the olfactory cleft and resected all tumor via endoscopic surgical approach. Histopathological diagnosis revealed that NCMH contained cartilaginous and mesenchymal components. In conclusion, NCMH is a rare surgically treated benign tumor that can be synchronously diagnosed with pleuropulmonary blastoma and should be kept in mind for differential diagnosis of unilateral pediatric nasal mass.
We report a typical case of nasal chondromesenchymal hamartoma (NCMH) of infancy, which extended to the orbit and intracranium through the skull base. The nasal mass was incidentally found in a 5-month-old otherwise healthy boy who had recently developed ptosis of the left eye. On neuroimaging, there were defects in the left ethmoid bone of the anterior cranial fossa and medial orbital wall. A round heterogeneous mass was found in the left nasal cavity. Solid portions were strongly enhanced. The preoperative diagnosis was nasal glioma or encephalocele. After frontal craniotomy, the mass was separated from the cranial basal dura. Through the transnasal approach, the mass was dissected from the left orbital content and removed. NCMH is a rare benign lesion and histopathological analogue of the mesenchymal hamartoma of the chest wall, having unique pathology: islands of hyaline cartilage, somewhat calcified or ossified hyalinized collagenous trabeculae and abundant myxoid stroma. Its occurrence in the nasal cavity was identified in 1998. The recognition of this entity is necessary to avoid misdiagnosis of the frozen section and for appropriate surgery.
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