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Case 284

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5-year-old male patient presents with left facial tumefaction and ipsilateral ptosis for the last 15 days. He denies fever, pain, headache or head trauma. His physical examination is remarkable for left ptosis, left orbital tumefaction with frequent eye tearing, but neither skin nor conjunctival hyperemia. Mouth deviation to the right, tongue deviation to the left and severe left visual acuity loss (capable of seeing only shadows).

Based on the clinical case and the images provided, what is the most likely diagnosis?

a) Orbital cellulitis

25%

b) Burkitt’s lymphoma

25%

c) Neuroblastoma

25%

d) Rhabdomyosarcoma

25%
   

Image analysis

 

Image 1: Non-contrast-enhanced head computed tomography (CT), axial sections at the level of the maxillary sinuses (A) and orbits (B). Soft tissue density expansive lesion in the left orbital and masticator spaces and ipsilateral pterygopalatine fossa, with extension to the nasal cavity and the adjacent maxillary and ethmoidal sinuses (yellow arrows).

 

Image 2: Contrast-enhanced head computed tomography (CT), axial sections at the level of the maxillary sinuses (A) and orbits (B).  Soft tissue density, contrast-enhancing expansive lesion in the left orbital and masticator spaces and ipsilateral pterygopalatine fossa, with extension to the nasal cavity and the adjacent maxillary and ethmoidal sinues (yellow arrows).

 

Image 3: Contrast-enhanced T1-weighted head magnetic resonance imaging (MRI), axial section at the level of the maxillary sinuses and nasal cavity. Infiltrative lesion in the masticator space with extension to the ipsilateral orbital and nasal cavities (white arrows).

 

Image 4: Contrast-enhanced T1-weighted head magnetic resonance imaging (MRI), coronal section at the level of the maxillary sinuses and orbits. Infiltrative lesion in the left masticator space (white arrows) with extension to the ipsilateral maxillary sinus (red arrow), orbit (red arrow) and nasal cavity (green arrow). 

Highlights

- Rhabdomyosarcomas are the most common malignant orbit tumors in children, especially those under 6 years old.

- Rhabdomyosarcomas present with painless, unilateral and rapidly progressive proptosis; edema and conjunctival hyperemia; extrinsic ocular motricity disorders and ptosis.

- The foremost important differential diagnosis to be excluded in cases of rhabdomyosarcoma is orbital cellulitis.

- Delay in diagnosis due to a rhabdomyosarcoma being mistaken for another cause of increase in orbital volume leads to poor outcomes.

- Treatment consists of surgery, chemotherapy and/or radiotherapy.

References

- Hoff, Paulo Marcelo Gehm et al. Tratado de Oncologia. 1st edition. São Paulo: Atheneu, 2012.

- Gappy, Christopher et al. Orbital cellulitis. UpToDate 2017 [Cited May 2017].

- Conneely MF, Mafee MF. Orbital rhabdomyosarcoma and simulating lesion. Neuroimaging Clin N Am 15 (2005), 121-136.

- Castillo, BV. Kaufman, L. Pediatric tumors of the eye and orbit. Neuroimaging Clin N Am 50 (2003) 149-172.

Author

Bruno Gonçalves Santos, 4th year medical student at UFMG (Federal University of Minas Gerais)

Mail: brunogsantos21[at]gmail.com

Supervisors

Dr. Karla Emília de Sá Rodrigues, professor of Pediatrics at Faculty of Medicine – UFMG

Mail: kesrodrigues[at]gmail.com

 

Dr. Nonato Mendonça Lott Monteiro, pediatric oncologist at Hospital das Clínicas da UFMG

Mail: nonatomlm[at]yahoo.com.br

Reviewers

Juliana Albano, Ricardo Mazilão, Wellerson Mayrink, Dr. Fernanda Teatini (neuroradiologist at Hospital das Clínicas da UFMG), Professor Viviane Parisotto and Dr. José Nelson Mendes Vieira (radiologist).

Translated by

Lucas Bruno Rezende, 5th year medical student at UFMG.

Test question

(HUPES – UFBA – Pediatric Oncology – 2014) Concerning rhabdomyosarcomas, select the correct alternative:

a) They are benign tumors that more frequently affect children under three years old

25%

b) They originate from skeletal striated muscle cells

25%

c) Rhabdomyosarcomas are notable for the fact that they always present with important inflammatory signs in the neighboring areas of the tumor

25%

d) Histologically, the embryonal subtype is the rarest

25%

e) Children under one year old have the best prgnosis, independent of other factors

25%
   

Commentics

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