A Case of Desmoid Fibromatosis in the Neck with

Spinal Neural Extension

Introduction:

In our local setting, there are a lot of patients presenting with neck
masses. Neck masses can have different pathology and consequent
different management. Thus it is important to determine the
histopathology of any tumor specially if with aggressive features
that warrant emergent management.

One of these soft tissue tumors is Desmoid tumor that is also known
as Desmoid Fibromatosis or aggressive fibromatosis. It is a rare
tumor with an incidence of 2-4 per million population and accounts
for 0.03% of all neoplasms. The peak age occurrence is about 30-40
years and occurs commonly in women compared to men.

This case report aims to present a case of Desmoid-type

fibromatosis on the left lateral neck with extension to the spinal Figure 1: 9 x 7cm firm, non-hyperemic, non-tender movable mass at the left
vertebrae, the diagnostic dilemma and the management done. lateral neck

A B

Figure 2: MRI findings showing T-weighted showing hypointense expansile left lateral neck mass (A) and T2-weighted showing
hyperintensity of the mass with close proximity to the left brachial plexus (B). Figure 3: Left spinal accessory nerve identified

Case:

A 44-year-old female had a 4-year history of gradually enlarging left lateral neck mass. A Neck CT scan with contrast and
MRI revealed a well-defined lobulated mass measuring 9.1 x 7.7 x 8.8 cm in the left lateral neck with T1-hypointense and T2-
hyperintense enhancement. An incision biopsy revealed fibrocollagenous and fibroadipose tissue with benign lymphoid
aggregates. A repeat deeper incision biopsy revealed a low-grade spindle cell neoplasm. Immunohistochemical staining
favored Desmoid-type fibromatosis. A multidisciplinary team conference discussed the plan of excision of left lateral neck
mass by ENT with excision of spinal neural extension C3-C4 by the Orthopedic service. The plan was carried out with
minimal residual tumor without any intraoperative problems. The final histopathology revealed low-grade spindle cell
neoplasm. Immunohistochemical staining revealed positive for S100, SOX10, Desmin, Caldesmon, and Beta-catenin
supporting Desmoid Type Fibromatosis. Post-operatively, the patient had limitation of abduction, adduction, flexion, and
extension of her left shoulder but she can shrug shoulders and move her left wrist with fine hand motor movements. She
underwent left upper arm and shoulder strengthening exercises. On subsequent check-up, the patient can minimally
abduct and adduct her upper arm but still cannot do flexion and extension of the elbows. Post-operative MRI monitoring
was carried out as suggested by the Radiation Oncologist.
Figure 4: Grossly, 10 x 9 cm hard, multilobulated mass on
the excised on the left lateral neck

Authors: Conclusion:
Gladys Jan V. Real, M.D Desmoid Fibromatosis in the Head and Neck is rare and definitive
Medical Officer III diagnosis is a challenge. In this case, Desmoid-type fibromatosis
occurred in the patient’s left lateral neck with extension to the
Samantha S. Castañeda, MD, FPSO-HNS vertebrae. Proper imaging modality was requested with a biopsy sent
for immunohistochemical staining to determine the diagnosis. A
Medical Specialist IV
multidisciplinary team approach was done to discuss the best plan for
the patient due to the aggressive extent of the tumor. Post-operative
Department of Otolaryngology-Head and Neck Surgery
care included every 6-months MRI monitoring of the residual tumor.
Rizal Medical Center, Philippines
Figure 5: Residual tumor on the level C2 foramina.