惡性 Schwannoma를 合倂한 von Recklinghausen氏病의 1例
A Mali후ant Schwannoma in von Recklinghausen's Disease
소속 상세정보
장은숙(張恩淑)/Eun Sook Chang
KMID : 0357919740080020153
Abstract
A mali후ant schwannoma is a relatively rare mali후ant neoplasm of nerve sheath
origin that locally infiltrates and also metastasizes, the intercellular matrix containing
collagen and mucin.
The diagnosis of malignant schwannoma should be primacy consideration in only two
following circumstances in the presence of a malignant tumor of soft tissue composed of
spindle cells :
1) when the tumor develops in patient with von Reklinghausen's disease, and
2) When tumor is obviously arising within the anatomic compartment of a major
nerve or in continuity with a unquestionable neurofibroma.
In the usual case, a plexiform neurofibroma is closely associated with a malignant
schwannoma. According to some investigators, notably Hosoi. the incidence of malignant
transformation of neurofibromatasis is as high as 13%.
The case reported herein is an example of malignant schwannoma to meet the above
criteria satisfactorily, grossly as well as microscopically.
This 42-year-old widow who is known to have von Recklinghausen's
neurofibromatosis for the past 10 years has developed a gradually growing subcutaneous
tumor in the volar surface of the left forearm with radiating and tingling pain in the
2nd and 3rd finger's of the left hand. The tumor had recurred three times artier local
excisions. This time it recurred 7 months after last excision.
A disarticulated left forearm was sent to laboratory which demonstrated the tumor to
be arising from the neurofibromatous median nerve and composed of large spindle cells
with whorl formation and organoid pattern. Individual cells show irregular
hyperchromatic nuclei and frequent mitotic figures.
About 2 months after amputation, a rapidly growing tumor on the left shoulder which
was diagnosed as neurofibrosarcoma elsewhere. And chest X-ray shows a diffuse
radio-opaque shadow on the right lung field which is suspected of a metastatic lesion.
Malignant schwannoma recurs as a rule and metastasizes at a late stage to fun by
blood stream and rarely to regional lymph node and centripedal metastasis to proximal
nerve. The treatment of choice is one stage radical surgery. And excisional biopsy for
diagnostic or therapeutic purpose is condemned. The prognosis usually is Poor.
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