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#1
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07-04-2024, 06:14 PM
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Neurofibromatosis
A 64-year-old lady with underlying neurofibromatosis presented with back swelling for the past five years. The swelling was painless, and it had rapidly increased in size for the past year with ulceration. She denied any fever; however, the ulcer was foul-smelling and associated with on-and-off pus discharge. However, she sought treatment for this problem multiple times and received antibiotics. She was treated for infected neurofibromatosis by a general practitioner. Due to an unresolved infection, she was referred to a tertiary center for further management. Upon review by the orthopedic team, there was a huge mass on her back with necrotic skin (Figures 1-2). The surrounding skin was erythema, and it was hard in consistency. She underwent a wide local excision of the mass. The intraoperative finding was an ulcerated soft tissue tumor measuring about 15 cm x 20 cm at the posterior trunk with central hemorrhagic necrosis (Figures 3-5). The mass is attached to the fascia and some parts of the erector spinae muscle. The mass was sent for histopathology examination (HPE). The HPE came back with a malignant peripheral nerve sheath tumor. Computed tomography (CT) scan of the thoracic, abdominal, and pelvis (TAP) showed multiple lung nodules and lytic bone lesions. She underwent adjuvant radiotherapy after surgery. She was doing well after surgery, as she was able to lie flat. Figure 6 shows her back condition two weeks after surgery. After six months of serial follow-up in the clinic, there was no sign of recurrent tumors.
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#2
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07-04-2024, 07:18 PM
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Re: Neurofibromatosis
I'm sometimes gobstruck when I see massive infected tumors with tissue necrosis that go virtually untreated for years without the patient succumbing to sepsis. As an aside though, modern dermatology has advancing treatments for many types of neurofibromatosis. A procedure using electrodesiccation and curettage can mostly dry up and remove those benign tumors on her skin, leading to a very improved quality of life. Here's an example photo...
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