LETTER TO THE EDITOR
Improvement of respiratory functions after excision of giant
neurofibroma of the chest wall
Selma Sönmez Ergün
Received: 20 March 2007 / Accepted: 20 July 2007 / Published online: 21 September 2007
Neurofibromatosis type I (NF-1) or Von Recklinghausen’s
disease is a hereditary neurocutaneous systemic disease
characterized by cafe au lait spots, generalized cutaneous
neurofibromas, skeletal deformities, central nervous system
tumors and disorders, somatic and endocrine abnormalities
Neurofibromas are the most common benign tumor of
NF-1. As they arise from perineural elements, neuro-
fibromas may occur in any part of the body. Common sites
include the face, the central nervous system, and the
skeletal system. Neurofibromas usually become symptom-
atic at an early age and exhibit progressive growth
throughout the patient’s life. They may affect the functions
of the surrounding anatomical structures and may also
cause cosmetic problems. Elephantiasis neurofibromatosa is
the most impressive manifestation of the disease due to its
size. In this setting, the lesion will lead to massive
overgrowth of both the skin and the associated soft tissue
and will cause disturbing deformities [1–3].
A 51-year-old male patient was admitted to our clinic
with slowly growing, mobile, pendulous soft tissue mass
extending from right pectoral region to the spine (Fig. 1).
Restriction of shoulder movements, respiratory compro-
mise, intertrigo, and the disturbing appearance of the chest
wall were main complaints of the patient. The pendulous
mass hung down to the waist measuring 50×70 cm causes
the chest wall deformity.
Diffuse cafe au lait spots in various sizes (diameter of six
spots >1.5 cm) were present in different parts of his body,
especially on the trunk, and also, there were freckles on his
axillary areas. The patient had kyphoscholosis and short
stature. Respiratory function tests demonstrated severe
restrictive type abnormalities [vital capacity (VC) 37% of
predicted; forced expiratory volume in one second (FEV1)
41% of predicted].
Ophthalmologic and other neurologic examinations were
normal. With these symptoms and findings, the diagnosis
was NF-1. The nearly total excision of the masses was
carried out under hypotensive general anesthesia.
The patient’s postoperative course was unremarkable,
and the resultant contour is satisfactory both for the patient
and for us.
Excision of all lesions in patients with neurofibromatosis
is not feasible; for that reason, surgical management is
reserved for functional or cosmetic problems. Surgical
excision does not fully treat the disease, but it can improve
the function and disturbing appearance as in our patient .
Although some reported cases had massive chest wall
infiltration, fortunately, our patient had only cutaneous
involvement [5, 6]. So we were able to resect the pendulous
tumor mass almost totally.
Eur J Plast Surg (2007) 30:149–150
S. Sönmez Ergün
Department of Plastic and Reconstructive Surgery,
Vak ıf Gureba Hospital,
Department of Chest Diseases, Vakıf Gureba Hospital,
S. Sönmez Ergün (*)
Bahçeşehir Emlak Bankası Konutları,
B 18 D3 C020403 34 900,
34900 Istanbul, Turkey