Fibrous Dysplasia of Skull: An Uncommon Presentation

Authors

  • Madhuri Sethia Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
  • Bushra Siddiqui Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
  • Mohd Rafey Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
  • Arshi Khan Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
  • Mahboob Hasan Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

DOI:

https://doi.org/10.21276/apalm.3553

Keywords:

Fibrous dysplasia, Monostotic, Polyostotic, MRI, Parietal lobe, Histopathological analysis

Abstract

Fibrous dysplasia is a developmental disorder of bone that can occur in monostotic or polyostotic form. Monostotic form is more frequent, usually seen in older children and young adults, commonly occurring in ribs, femur, tibia and craniofacial bones. Polyostotic fibrous dysplasia is less common, affecting as few as two bones to as much as 75% of the skeleton, predominantly involving the femur, pelvis and foot. We present a case of fibrous dysplasia involving the skull in a 29-year-old woman. On magnetic resonance imaging (MRI), a well-defined lytic lesion was identified in the outer cortex of the left parietal lobe for which two differential diagnoses were made: Langerhans cell histiocytosis (LCH) and lymphoma. The patient underwent craniotomy followed by mesh cranioplasty. The atypical location of the lesion initially obscured the diagnosis, which was ultimately confirmed through histopathological analysis.

References

1. Riddle ND, Bui MM. Fibrous dysplasia. Arch Pathol Lab Med. 2013 Jan 1;137(1):134-8.

2. Feller L, Wood NH, Khammissa RA, Lemmer J, Raubenheimer EJ. The nature of fibrous dysplasia. Head Face Med. 2009;5:1-5.

3. Parekh SG, Donthineni-Rao R, Ricchetti E, Lackman RD. Fibrous dysplasia. J Am Acad Orthop Surg. 2004;12:305-13.

4. DiCaprio MR, Enneking WF. Fibrous dysplasia: pathophysiology, evaluation, and treatment. JBJS. 2005;87:1848-64.

5. Chapurlat RD, Meunier PJ. Fibrous dysplasia of bone. Best Pract Res Clin Rheumatol. 2000;14:385-98.

6. Riminucci M, Fisher LW, Shenker A, Spiegel AM, Bianco P, Robey PG. Fibrous dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation. Am J Pathol. 1997;151:1587.

7. Pham TA, Nguyen DH, Ngo HT, ThuyLe MA. Fibrous Dysplasia of the Parietal Bone with Focal Motor Seizures: A Case Report. Am J Case Rep. 2024;25:e943718-1.

8. Mahajan S, Kamboj M, Baoz K. Maxillofacial fibrous dysplasia. Indian J Dent Res. 2005;16:151-2.

9. Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg. 1938;36:874-98.

10. Mohan H, Mittal P, Mundi I, Kumar S. Fibrous dysplasia of bone: a clinicopathologic review. Pathol Lab Med Int. 2011;6:31-42.

11. Lustig LR, Holliday MJ, McCarthy EF, Nager GT. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg. 2001;127:1239-47.

12. Glotzbecker MP, Carpentieri DF, Dormans JP. Langerhans cell histiocytosis: Clinical presentation, pathogenesis, and treatment from the LCH etiology. Univ Pa Orthop J. 2002;15:67-73.

13. Bhagavathi S, Wilson JD. Primary central nervous system lymphoma. Arch Pathol Lab Med. 2008;132:1830-4.

Downloads

Published

20-08-2025

How to Cite

1.
Sethia M, Siddiqui B, Rafey M, Khan A, Hasan M. Fibrous Dysplasia of Skull: An Uncommon Presentation. Ann of Pathol and Lab Med [Internet]. 2025 Aug. 20 [cited 2025 Dec. 5];12(8):C110-C112. Available from: https://pacificejournals.com/journal/index.php/apalm/article/view/3553

Issue

Section

Case Report