nasales chondromesenchymales Hamartom

Nasal

chondromesenchymal hamartoma in young children: CT and MRI findings and review of the literature. CT of sinonasal cavity. (a) An axial non-contrast CT revealed an ovoid soft-tissue mass in the right nasal cavity. (b ) An axial contrast-enhanced CT image showed marked enhancement. (c) A coronal contrast-enhanced CT image revealed a marked enhanced mass which extends into the anterior cranial fossa and erodes the cribriform plate (arrow). The nasal septum was deviated to the left and the right nasal cavity dilated.

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Nasal

chondromesenchymal hamartoma in young children: CT and MRI findings and review of the literature. MR Imaging of sinonasal cavity. (a) An axial T1-weighted MRI revealed a mildly hypointense mass in the right nasal cavity. (b) An axial T2-weighted MRI showed a well-defined hyperintense mass and strong signal in both maxillary sinuses. (c) A coronal contrast-enhanced T1-weighted MRI showed striking enhancement of the mass, extending into the anterior fossa with intact dura.

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Nasal

chondromesenchymal hamartoma in young children: CT and MRI findings and review of the literature. An axial non-contrast CT revealed an well-defined, irregular, calcified soft-tissue mass in the left nasal cavity.

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Nasal

chondromesenchymal hamartoma in young children: CT and MRI findings and review of the literature. MR Imaging of sinonasal cavity. (a) An axial T2-weighted MRI showed a well-defined, heterogeneous hyperintense mass containing multiple small round areas of higher signal intensity (arrow). (b) Axial contrast-enhanced fat-suppressed T1-weighted image demonstrated striking heterogeneous enhancement of the mass with multiple non-enhanced cystic components within the lesion (arrow).

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Chondromesenchymal

hamartomas in a 24-year-old male mimicking a posterior mediastinal tumor and a 5-month-old boy with postoperative disseminated intravascular coagulation: two case reports. Radiological appearance of chondromesenchymal hamartoma of the chest wall in a 24-year-old adult. a, b DR suggested a left posterosuperior mediastinal mass and bronchitic changes. c CT revealed a benign expansile lesion in the posterior part of the left fifth rib with interior punctate calcifications. d-f MRI revealed a well-circumscribed expansile heterogeneous soft mass closely adjoining the fifth vertebral body in the left posterior mediastinum. DR, digital radiography; CT, computed tomography; DR, digital radiography; MRI, magnetic resonance imaging

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Chondromesenchymal

hamartomas in a 24-year-old male mimicking a posterior mediastinal tumor and a 5-month-old boy with postoperative disseminated intravascular coagulation: two case reports. Imaging findings of chondromesenchymal hamartoma of the chest wall in a 5-month-old infant. a DR revealed a soft tissue mass in the left middle lung field, accompanied by collapse of the adjacent thoracic cage. b DR revealed postoperative loss of parts of the left fifth and sixth ribs. c, d CT revealed a benign solid-cystic lesion with multiple speckled and cord-like high-density shadows, arising from the axillary segment of the left fifth and sixth ribs; the corresponding cortical and medullary cavities of the ribs were involved. DR, digital radiography; CT, computed tomography

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Chondromesenchymal

hamartoma • Sinonasal chondromesenchymal hamartoma - pediatric - Ganzer Fall bei Radiopaedia

Walters C, Sinonasal chondromesenchymal hamartoma - pediatric. Case study, Radiopaedia.org (Accessed on 18 Nov 2023) https://doi.org/10.53347/rID-171799 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

Chondromesenchymal hamartomas are rare, benign, tumor-like nasal masses in children that have been associated with DICER1 mutations.

Epidemiology

The entity is rare: a systematic review of the literature in 2015 identified fewer than 50 reported cases . The mean age of presentation is 10 years .

Clinical presentation

Patients most commonly present with nasal congestion or mass on examination .

Pathology

Location

The mass is typically centered in the nasal cavity but commonly also involves the paranasal sinuses, orbit, and/or anterior skull base .

Classification

Chondromesenchymal hamartoma was introduced in the World Health Organization classification of head and neck tumors, 4 edition (2016), under the category "Tumors of the nasal cavity, paranasal sinuses, and skull base."

Microscopic appearance

Histology shows nodular hyaline cartilage with a spindle cell stroma .

Genetics

Chondromesenchymal hamartoma is associated with somatic or germline DICER1 mutations . It is therefore part of the phenotypic spectrum of DICER1 tumor predisposition syndrome.

Radiographic features

The imaging appearance is non-specific, consisting of an expansile, circumscribed sinonasal mass .

CT

CT demonstrates polypoid soft tissue in the nasal cavity. There may be osseous erosion, internal calcifications, and cystic components .

MRI

The signal characteristics reflect myxoid stroma with low cellularity and variable vascularity :

T1: isointense to brain
T2: heterogeneously hyperintense, with some cystic areas approaching cerebrospinal fluid intensity
T1 C+: variable enhancement