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| Year : 1985 | Volume
: 31
| Issue : 1 | Page : 59-61 |
Intra-osseous neuro-fibrosarcoma (a case report).
Yadav SS
How to cite this article:
Yadav S S. Intra-osseous neuro-fibrosarcoma (a case report). J Postgrad Med 1985;31:59-61 |
Neurogenic tumours arising from the bone are rare. Only isolated cases of such lesions have been reported; these tumours are called by many names such as neurofibroma, neurinoma, schwannoma, perineural fibroblastoma, neurogenic sarcoma, malignant schwannoma and neurofibrosarcoma.[1],[6] Peer[8] described an intramedullary neurogenic sarcoma of the shaft of the ulna which resulted in an amputation. Guthert[5] reported a malignant neuroma in the mid-shaft of the humerus, which had cortical perforation. We intend to report a rare case of intramedullary neurofibrosarcoma of the femur. The occurrence of such a lesion, together with the surgical excision of the tumour, forms the basis of this report.
A 27 year old woman complaining of a gradually increasing, dull ache is the right groin and inability to walk for the past 3 months, was examined on 20eth August 1930. She denied any history of fever or trauma. Physical examination revealed clinical evidence of generalised neurofibromatosis. There was tenderness over the right groin and the trochanteric region. The movements of the right hip were painful and grossly restricted. However, there was no limb length discrepancy. While in the hospital, she complained of pain over the medial aspect of the right thigh in its lower third. Roentgenographic examination, which included a skeletal survey, revealed a large osteolytic lesion in the proximal end of the right femur involving the trochanteric region and the neck with a pathological fracture [Fig. 1A]. A femoral arteriogram revealed evidence of hypervascularity. A small osteolytic area was observed in the same femur, an inch above the medial condyle [Fig. 2]. The chest and the abdominal roentgenograms were considered to be within normal limits.
Results of indicated laboratory investigations were as follows: Haemoglobin was 8.5 gm%; TLC was 7000 per cmm with N-64%, E-8% and L-28%. ESR was 125 mm (first hour Westergren). Blood urea was 25 mg% and sugar, 99 mg%. Serum calcium was 10 mg%, serum phosphorus, 3.5 mg% and alkaline phosphatase, 12.5 K.A. units. Serum creatinine was 0.7 mg%. VDRL was negative. Urine examination was within normal limits.
On 16th September 1980, an open biopsy was performed from the trochanteric area on the right side which revealed evidence of neurofibrosarcoma [Figs. 3A] and [Figs. 3B]. Histopathological report from the distal lesion was suggestive of neurofibroma [Figs. 4A] and [Figs. 4B] .
Since pain in the right groin was gradually increasing, excision of the proximal end of the right femur was performed on 25th September, 1980. Due to non-availability of a homologous replacement and a long stem prosthesis, the resultant gap was bridged by a double fibular grafting along with cancellous chips from the iliac crest [Fig. 1B]. At the same sitting. the small lytic area in the supracondylar region was thoroughly curetted and filled with cancellous bone chips [Fig. 2B]. The patient was encased in a hip spica. The post-operative period was uneventful. On the 15th post-operative day, stitches were removed through a window made in the POP cast and the patient was discharged from the hospital.
On 7th February 1981, she was re-admitted for pain in the neck and progressive weakness of the left upper limb. There was a 4" x 4", firm and tender swelling related to the left lobe of the thyroid. Roentgenograms of the cervical spine were within normal limits. She did not want any more surgery and left the hospital at request on 21st February 1981. She later died at home on 5th March 1981.
The specific nerve tumour in the bone occurs on rare occasions. In majority of the reported cases, these tumours have involved the mandible, apparently because it contains a large segment of the mandibular nerve.[3] However, such lesions often develop subperiosteally and erode the bone from outside.[2],[7] Stout,[9] in a survey of nearly 246 cases of peripheral nerve sheath tumours, did not mention a single instance of intra-osseous involvement. In the earlier reported cases,[2],[4],[8] the tumour had usually affected the mid-shaft of the long bones. In our case, however, both the lesions involved the proximal and distal metaphyseal regions of the same femur. The proximal lesion had been growing fast and was diagnosed as neurofibrosarcoma.
True involvement of bone in Von Recklinghausen's disease is rare and of vital importance.[2] Out of 7 cases in a series of 3987 proved, primary, bone tumours reported from The Mayo Clinic, only one patient had completely intraosseous neurofibroma of the mandible with Von Recklinghausen's neurofibromatosis.[3] The rarity of neurofibrosarcoma arising in the bone is puzzling and is rarely reported in the course of generalised neurofibromatosis.[6] Pathological fracture may occur due to extensive intra-osseous involvement. In the present case, the lesion in the proximal end of the right femur was malignant and had led to a pathological fracture of the neck of the femur [Fig. 1]. On a clinical and roentgenological evaluation, the osseous lesions were thought to be one of the giant cell variants and the true nature of the lesion could be determined only by a histopathological examination.
A sharp and well defined roentgenographic appearance indicates the benign nature of the lesion.[3] The benign lesion involving the lower end of the femur in the present case supports this view. A through histological examination, however; is essential as the cells, by metaplasia, may produce a wide variety of tissues, e.g., cartilage, bone, fat etc., not ordinarily thought to be derived from them.[3] It is possible that with the remarkable versatility of the cells, such a neoplasm may even go unrecognised.[7]
The management of each case must be decided on its own merits. Although local excision is considered adequate in benign lesions, an en block resection or amputation is advocated in malignant lesions. In the present case, the neurofibrosarcoma of the proximal end of the femur was excised en block and the gap was bridged by a double fibular grafting. The other benign lesion involving the lower end of the femur was curetted and was packed with cancellous bone chips.
The author is grateful to the Director, JIPMER, Pondicherry, for his kind permission to publish this rare report.
| 1. | Conley, A. H. and Miller, D. S.: Neurilemmoma of bone; A case report. J. Bone and Joint Sug., 24: 684-689, 1942.  |
| 2. | de Santo, D. A. and Burgess, E.: Primary and secondary neurilemmoma of bone. Surg. Gaynaec. and Obstet., 71: 454-461, 1940. |
| 3. | Fawcett, K. J. and Dahlin, D. C.: Neurilemmoma of bone. Amer. J. Clin. Pathol., 47: 759-766, 1967. |
| 4. | Gross, P., Bailey, F. R. and Jacox, H. W.: Primary intramedullary neurofibroma of the humerus. Arch. Pathol., 28: 716-718, 1939. |
| 5. | Guthert, H.: Ein Malignes Neurinom des Knochen: Zentralble fur Allgemine Pathologic and Pathologische Anatomic, 88: 185-188, 1952. Quoted by Lichtenstein. |
| 6. | Hart, M. S. and Basom, W. C.: Neurilemmoma involving bone. J. Bone and Joint Surg., 40A: 465-468, 1958. |
| 7. | Lichtenstein, L.: Tumours of nerve origin. In, "Bone Tumours". 5th Edition, the C. V. Mosby Company, Saint Louis, 1977, p. 181. |
| 8. | Peers, J. H.: Primary intramedullary neurogenic sarcoma of the ulna. Report of a case. Amer. J. Pathol., 10: 811820. 1934. |
| 9. | Stout, A. P.: Peripheral manifestations of the specific nerve sheath tumour (neurilemmoma), Amer. J. Cancer, 24: 751-796, 1935. |
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