Pulmonary metastases - skull round cell tumor primary

Case contributed by Dr Mostafa Mohamed


Fever and cough with known malignant scalp swelling

Patient Data

Age: 5 years
Gender: Female

Multiple bilateral variable-sized pulmonary nodules are noted with the largest of them being para hilar and measuring 5.2 x 3.7 cm on the right side and 4.9 x 3.8 cm on the left side. Other smaller nodules are seen with all of them showing the angiocentric sign denoting their hematogenous origin. No extra thoracic extension. Some of the nodules are surrounded by ground-glass attenuation, halo sign.

Right temporoparietooccipital mass that erodes the skull and shows intracranial extension surrounded by vasogenic edema and exerts a mass effect in the form of compression of the posterior horn of right lateral ventricle and midline shift. 

Postoperative CT shows the resection of the extracranial portion of the tumor with the persistence of the intracranial part due to intraoperative bleeding.

Scalp mass which proven by pathology to be malignant round cell tumor.

Case Discussion

This is a case of extensive pulmonary metastases that is accidentally discovered by a postoperative CT chest that is done due to chest infection. It shows an angiocentric sign which refers to the vessel directed toward nodule feeding it denoting hematogenous spread. Halo sign in pulmonary metastasis is due to the fragility of neovascular tissue leading to rupture of the vessel.

The Patient undergoes brain surgery but unfortunately, only the extracranial part was excised due to bleeding.  

Pathology of the primary tumor:

Gross picture: A soft grayish-white pink mass measured 7 x 6 cm together with smaller tissue pieces admixed with bony fragments.

Microscopic picture: Highly cellular tumor tissue, made up of diffuse monotonous infiltration by neoplastic small round blue cells, with areas of necrosis and dense desmoplastic stroma. The cytoplasm was scant, granular, and eosinophilic. The nuclei showed granular with irregularly distributed chromatin. soft tissue and bone infiltration were present.

Conclusion: Right temporoparietooccipital SOL, excision biopsy:

Round blue cell neoplasm for immunophenotyping. 

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