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Neurorradiologia

19/09/2024
Da Redação

Clinical History

Female.
23 years old.
From Aparecida de Goiânia – GO.
No history of previous illnesses.
The patient was admitted with a mass on the left hemicranium, which has progressively grown over the past 4 years.
In recent months, the mass has been accompanied by increasing pain and headaches.
Previous investigations with laboratory tests did not reveal any abnormalities.

Images

Physical Examination

Large, expansile mass on the left hemiface causing distortion of facial structures, including the ipsilateral eye and ear. The lesion shows two necrotic regions: one anteriorly and the other laterally.
Fibroelastic consistency with focal areas of induration, tender on palpation, with anterior discharge of sanguineous fluid upon compression.

Diagnostic Imaging

Computed tomography

Contrast-enhanced axial CT

Soft tissue density mass, involving the superficial layers of the left hemiface, apparently originating from the cutaneous and subcutaneous tissues.
The mass is composed of nodular material with heterogeneous attenuation. The nodules have hyperdense contours and predominantly hypodense internal contents with heterogeneous enhancement.

Irregularity in the cranial skull with thinning and remodeling.
There was no intracranial extension.

Contrast-enhanced axial CT (arterial phase)

Diagnostic imaging of the chest and abdomen revealed no additional abnormalities.

Magnetic resonance imaging

Multiloculated mass with high signal intensity on T2WI/FLAIR, with flow voids

High / intermediate signal intensity on T1WI (*),

Restricted diffusion within the mass (*).

Areas of low signal intensity on T2* imaging (*).

Peripheral and heterogeneous enhancement, delineating multiple regions of necrosis (red) | There is an expansile effect in the left orbit (blue)

Differential Diagnosis

Peripheral Nerve Sheath Tumor – Benign

Peripheral Nerve Sheath Tumor – Malignant

Dermatoﬁbrosarcoma Protuberans

Others

Case Outcome

Aggressive surgical resection was performed

General macroscopic appearance of the tumor after surgical resection.
Weighing 2500g and measurie 25.0 x 12.0 x 10.0 cm.

Hypoechoic nodules with debris and echogenic foci.
Surrounded by an isoechoic stroma.

Gross anatomy exhibits a multinodular appearance (blue), nodules with a firm-elastic consistency (green) reddish/hemorrhagic spots (red).
Areas with a friable, trabeculated consistency and cystic cavities, areas of tumor necrosis (black).

Histopathological and immunohistochemical studies

Leia a discussão do caso

Malignant Peripheral Nerve Sheath Tumor (MPNST)

Ricardo Vieira Teles Filho | Hospital das Clínicas – Federal University of Goiás (HC-UFG)

Sarcoma with peripheral nerve sheath differentiation typically has aggressive behavior. Can occur in the following settings:

Sporadic (~ 50%).
In neurofibromatosis type 1 (40-50%).
In the setting of prior radiation therapy (10%).

Can arise in virtually any anatomic location. The most common sites are the trunk and extremities, followed by head and neck. No sex predilection. Diagnosis:

Histologic evaluation is necessary AND requires correlation with radiologic findings.

Treatment:

Surgical resection followed by radiation therapy to achieve local control.
Therapeutic options for metastatic MPNST are limited
Conventional chemotherapy is typically reserved for patients with metastatic disease.
A dramatic response to vemurafenib has been reported in a single case harboring BRAF V600E mutation.

Prognostic factors:

Aggressive behavior, including metastatic disease and local recurrence, may occur.
Tumors located on the trunk have a worse prognosis compared to those on the extremities.
Patients with NF1 and radiation-associated MPNST have a poorer prognosis.

Case follow-up

Aggressive surgical resection was performed with complete removal of the lesion, followed by reconstruction by the plastic surgery team. The surgery lasted a total of 4.5 hours and proceeded without complications.
The patient remained in the ICU for 2 days post-surgery without complications. She was discharged from the hospital after 10 days and was asymptomatic. She began radiotherapy and continues with oncological follow up in conjunction with plastic surgery, remaining without complications.
A follow-up contrast-enhanced CT scan was performed, revealing no signs of intracranial or local complications postoperatively and no evidence of residual tumor.
The challenge in diagnosing Malignant Peripheral Nerve Sheath Tumor (MPNST) lies in the essential integration of radiological studies and histopathological analysis. Joint discussion and follow-up are crucial to achieving a definitive diagnosis and ensuring appropriate patient management.

Bibliography

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