Table 1.

Clinical Details of Head and Neck Fibromatosis Study Group.

Patient # / Site Symptoms Structures Involved Treatment Modality PresentStatus Follow-up (Months)
#1 / Neck Pain, left neck swelling, left upper limb motor deficit Left paravertebral C4–C7 levels Surgery + chemotherapy + hormonal therapy Stable disease 77
#2 / Neck, supraclavicular fossa Swelling over left supraclavicular fossa Left brachial plexus with encasement of vertebral artery Surgery + chemotherapy + hormonal therapy Stable disease 32
#3 / Mandible Swelling below right lower jaw Right lower gingivo-buccal sulcus, floor of mouth, involving strap muscles but not the thyroid cartilage Surgery + chemotherapy + hormonal therapy Disease regression 31

#4 / Neck Right hand swelling, tingling and numbness Lesion extending from C4–D2 and extension into C5–C6 nerve roots encasing subclavian artery Metronomic chemotherapy Stable disease 1*
#5 / Neck, supraclavicular fossa Swelling over left supraclavicular fossa Loss of fat planes in superior trunk of brachial plexus Metronomic chemotherapy Stable disease 56

#6 / Posterior neck Swelling in left posterior side of neck Left posterior spine region Surgery + metronomic chemotherapy + hormonal therapy Disease regression 41

#7 / Posterior neck Swelling in posterior side of neck Nape of neck, vertebral artery, paraspinal muscles Surgery + adjuvant radiotherapy Disease regression 29

#8 / Thyroid Swelling, change in voice, difficulty swallowing Large lobulated mass in retropharyngeal region, tracheal compression and displacement Surgery + radiotherapy + chemotherapy + hormonal therapy Disease regression 21
#9 / Maxilla Pain, swelling over left side of face, vision loss, ear discharge, nasal blockage Intracranial extension Surgery + radiotherapy + chemotherapy + hormonal therapy Disease progression 4

#10 / Left supraclavicular fossa Swelling in left supraclavicular region, left shoulder pain radiating to left arm Left supraclavicular fossa lesion with loss of fat planes in relation to left common carotid artery and subclavian artery Chemotherapy + hormonal therapy Stable disease 14

#11 / Left supraclavicular fossa Swelling in left supraclavicular region Brachial plexus lesion encasing the common carotid artery Surgery + radiotherapy (defaulted) + metronomic chemotherapy Stable disease 9
*This patient experienced neck swelling for 7 years, which was progressive in nature. She presented with compressive symptoms but was inoperable. The patient received metronomic chemotherapy for 1 month but was lost to follow-up after that.
RMMJ Rambam Maimonides Medical Journal Rambam Health Care Campus 2021 July; 12(3): e0022. ISSN: 2076-9172
Published online 2021 July 20. doi: 10.5041/RMMJ.10444