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Multicentric infantile myofibromatosis: a rare disorder of the calvarium

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Abstract

Introduction

Infantile myofibromatosis is a rare mesenchymal disorder that occurs predominantly in infancy and early childhood, in either solitary or multicentric form. It can affect soft tissue, muscle, skeleton, and occasionally, visceral organs. Infantile myofibromatosis without visceral involvement frequently undergoes spontaneous regression. Multicentric infantile myofibromatosis with involvement exclusively of the calvarium is extremely rare.

Discussion

We report an 8-month-old girl who presented with multifocal calvarial lesions. The child underwent total excision of the temporal mass, and histopathological study gave a diagnosis of infantile myofibromatosis. Serial follow-up by neuroimaging was obtained at 3, 6, 12, and 24 months postoperatively. Three months after surgery, a new lesion in the midline of frontal bone was found, and there was partial regression of the occipital lesion. Complete regression of the untreated lesions was shown at 24 months. Illustrated by our patient and literature review, we emphasize the importance of recognition and proper intervention for this rare, nonmalignant disorder.

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Authors and Affiliations

  1. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

    Da-Biao Zhou, Ji-Zong Zhao & Dong Zhang

  2. Department of Pathology, Beijing Jishuitan Hospital, The 4th Clinical College of Peking University, Beijing, China

    Xiao-Yuan Huang

Authors
  1. Da-Biao Zhou
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  2. Ji-Zong Zhao
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  3. Dong Zhang
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  4. Xiao-Yuan Huang
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Corresponding author

Correspondence to Da-Biao Zhou.

Additional information

Comment

The case is well imaged and I found the review of historical cases useful to confirm the attitude to a “wait and see” policy for multiple (solitary) skull lesions after diagnosis, a thing that also a first reader on this subject will find helpful for the management of occasionally observed cases.

Gianpiero Tamburrini,

Rome, Italy

Comment

The rarity of this pathological entity, the particular multifocal location to the calvaria justifies its knowledge.

It is important to recognize this lesion from the differential diagnosis with others pathological entities as eosinophilic granuloma, fibrosarcoma, fibrous dysplasia, epidermoid cyst, diploic meningioma, osteomyelitis.

The authors have done a exhaustive revision of literature and the discussion about the strategy to choose the treatment of this non malignant pathology is important to avoid surgical extensive removal for a disease that can regress spontaneously because it is a self-limited disorder.

If the calvaria location is very rare as single location, the intraparenchymal extension is exceptional as reported in literature.

The natural history is characterized by a period of initial rapid growth followed by a stabilisation and a spontaneous regression.

The role of surgery is admitted to take a sample to establish a true diagnosis mainly in solitary lesion.

The paper is well written and well documented and so can help all neurosurgeons to think of this lesion in case of pathological disease of the calvaria either in a single or in multiple locations.

Carmine Mottolese,

Lyon, France

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Zhou, DB., Zhao, JZ., Zhang, D. et al. Multicentric infantile myofibromatosis: a rare disorder of the calvarium. Acta Neurochir 151, 641–646 (2009). https://doi.org/10.1007/s00701-009-0264-y

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  • DOI: https://doi.org/10.1007/s00701-009-0264-y

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