Br. J. Surg. Vol. 62 (1975) 573-575
Nodular fasciitis : an unusual cause of extradural spinal cord compression R . P . S E N G U P T A , S . C. SO A N D R . H . P E R R Y * SUMMARY
A case of nodular fasciitis causing extradural spinal cord compression with a successful outcome following removal is reported. Such a tumour within the spinal cord has never been reported. The pathogenesis of a nodular fasciitis in this situation and its differential diagnosis is discussed. NODULAR fasciitis is a well-recognized tumour occurring in the subcutaneous tissues over the trunk and limbs but has never been observed in the spinal canal. The following is a case report of such a tumour arising in the extradural space and causing spinal cord compression. Case report A 64-year-old retired schoolteacher was admitted to the Regional Neurological Centre in January 1974 with a I-month history of lower thoracic back pain. It was constant and radiated to the right hypochondrium. Three weeks before admission he had noticed that he was unsteady on walking and
Fig. 2. Photomicrograph illustrating marked vascularity of the edge of the lesion. Arrows point to vascular spaces. HE. ( x 160.)
that his legs had become weak. Shortly afterwards he developed hesitancy and urgency of micturition. Two days before admission he was unable to walk. For the past 8 years he had had hypertension and 2 years previously had had a cerebrovascular accident causing a left hemiparesis. On examination the patient had a localized tenderness over the spine of D9. Marked weakness was present in both lower limbs involving all muscle groups. There was no wasting, the tone was increased with brisk reflexes and the plantar responses were extensor. Pain and temperature sensation below the level of D9 were impaired. Both position and vibration sensations were absent below the hip joint and Romberg’s sign was positive. He was hypertensive (220/130 mm Hg) with retinal vascular changes and left ventricular voltage changes o n the ECG. Plain X-rays of the spine were normal. Myelography showed a complete block a t D9 with features suggesting a n extradural tumour. The cerebrospinal fluid protein was 76 mg per cent. A t operation the laminae from D8 to D11 were removed. A firm lobdated tumour was found in the extradural space pushing the cord anteriorly. The tumour was not attached to the dura or any nerve root. It was only loosely attached to Fig. 1. Photomicrograph illustrating a haphazard cellular arrangement with reticulin fibres outlining cells and demarcating vascular spaces. Reticulin. ( x 120.)
* Department of Neurosurgery and Neuropathology, Regional Neurological Centre, Newcastle General Hospital, Newcastle upon Tyne. 573
R. P. Sengupta et al.
Fig. 3. Photomicrograph showing vascular clefts and intercellular spaces, many of which contain red blood cells. HE. ( X 160.) fibrofatty tissue and required little dissection for complete removal. The patient made an uneventful recovery from the operation. Over a period of 4 weeks the power in the lower limbs returned to almost normal. The sensory deficit also recovered to a large extent and at the time of his discharge 4 weeks later he was able to walk unaided. Histological examination revealed a circumscribed cellular nodule measuring 3.8 x 2.5 x 1.5 cm attached a t one point to vascular fibrous fatty tissue. An incomplete fibrous capsule partially defined the edge of the nodule. Microscopic examination revealed the predominant cell type to be fibroblastic with vesicular lightly staining nuclei and eosinophilic cytoplasm. In the central region the cells were either plump and ovoid with an ill-defined eosinophilic cytoplasm and irregular reticulin fibres (Fig. I), or spindle-shaped with a compact fasciculated pattern and a regimented arrangement of reticulin fibres. In the peripheral regions the cells were less compact. Only occasional mitotic figures were identified and none had abnormal features. The stroma was highly vascular, particularly at the periphery (Fig. 2), with many vascular clefts, and spaces separated the fibroblastic cells. Throughout the tumour red blood cells lay in endothelialized clefts and non-endothelialized intercellular spaces (Fig. 3). Occasional vessels were cuffed with sparse accumulations of lymphoid cells. A small amount of collagen was present; no multinucleated cells were identified. Apart from the fat cells at the edge of the nodule no lipidcontaining cells were identified to suggest that the tumour had a lipomatous origin and the nodule had no features distinctive of a neurilemoma (no Antoni tissue type A and B and palisading).
Discussion The term ‘nodular fasciitis’ is now generally accepted as an apt description of a peculiar lesion which is 574
usually found in subcutaneous or deep fascia1 planes (Leading Article, 1973). The nodule is generally 1-3 cm in size and has distinctive histological features. It appears as a vascular proliferation of fibroblasts with a patchy lymphocytic infiltration, occasional islands of collagen and occasional giant or multinucleated cells (Price et al., 1961; Mackenzie, 1970). The cellularity of the lesion and the presence of mitotic figures have led to it being confused with a variety of mesodermal tumours and reactive processes, including sarcomas. It usually grows rapidly but it does not metastasize or recur if completelyexcised (Stout, 1961; Hutter et al., 1962). Although known to occur in a variety of locations. including the orbit (Font and Zimmerman, 1966), it has not to our knowledge been described as an extra, dural tumour causing compression of the spinal cordAnalysis of spinal cord tumours in the past (Elsberg, 1941; Kernohan, 1941; Austin, 1961) has listed ‘fibromata’ as constituting less than 1 per cent of extradural neoplasms. Unfortunately the histology of these fibromata was not described. The origin of nodular fasciitis is still in doubt. Traumatic stimulus has been cited as a possible aetiological factor (Soule, 1962), but in the present case there was no history of trauma to the back. The unusual situation in this case raises the questions of dermal implantation (Manno et al., 1962; James and Lassman, 1972), but a lumbar puncture had not been performed previously. In subcutaneous tissues nodular fasciitis is relatively harmless, but in this case in the confines of the vertebral canal it caused spinal cord compression and paraplegia. It is important to identify the lesion histologically because, despite its appearance, it is benign (Price et al., 1961; Hutter et al., 1962; Soule, 1962), and in the present case its removal at an early stage has prevented a permanent neurological deficit.
Acknowledgements The authors wish to thank Professor John Hankinson under whom this patient was admitted, and Professor B. E. Tomlinson for verifying the histology of the tumour. References (1961) The Spinal Cord. Springfield, 111. Thomas, pp. 104-105. ELSBERG c. A. (1941) Surgical Diseases of the Spinal Cord, Membranes and Nerve roots. New York, Hoeber, p. 49. FONT R . L. and ZIMMERMAN L. E. (1966) Nodular fasciitis of the eye and adnexa. Arch. Ophthalmol. 75, 475-48 1. HUTTER R. V. P., STEWART F. W. and FOOTE F. W. (1962) Fasciitis. Cancer 15, 992-1003. JAMES c. c. M. and LASSMAN L. P. (1972) Spinal Dysraphism. London, Butterworths, pp. 18-24, 57-60. KERNOHAN J. w. (1941) Tumours of the spinal cord. Arch. Pathol. 32, 843-883. AUSTIN G.
Nodular fasciitis LEADING ARTICLE
(1973) Nodular fasciitis. Lancet 1,
870. (1970) The Diflerential Diagnosis of Fibroblastic Disorders. Oxford, Blackwell, pp. 18-27. MANNO N. J., KIHLEIN A. and KERNOHAN J. w. (1962) Jntraspinal epidermoids. J. Neurosurg. 19, 754765. MACKENZIE D. H.
PRICE E. B. jun., SILLIPHANT w. M. and SHUMAN R.
(1961) Nodular fasciitis : a clinico-pathological analysis of 65 cases. Am. J. Clin. Pathol. 35, 122-136. SOULE E. G. ( I 962) Proliferative (nodular) fasciitis. Arch. Pathol. 73, 4 3 7 4 . STOUT A. P. (1961) Pseudosarcomatous fasciitis in children. Cancer 14, 1216-1222.
Errata British Journal of Surgery, Vol. 62: No. 3 : March 1975, p. 195. The legends to Figs. 2, 4 and 5 should read as follows: Fig. 2. Photomicrograph of regenerating liver at 72 hours . . . . Fig. 4. Case 7. Rise in serum bilirubin level after hepatic resection with unconjugated fraction as the main component (type I). Fig. 5. Case 6. Rise in serum bilirubin level after hepatic resection with the conjugated fraction as the main component (type 11).
575
Nodular fasciitis : an unusual cause of extradural spinal cord compression R . P . S E N G U P T A , S . C. SO A N D R . H . P E R R Y * SUMMARY
A case of nodular fasciitis causing extradural spinal cord compression with a successful outcome following removal is reported. Such a tumour within the spinal cord has never been reported. The pathogenesis of a nodular fasciitis in this situation and its differential diagnosis is discussed. NODULAR fasciitis is a well-recognized tumour occurring in the subcutaneous tissues over the trunk and limbs but has never been observed in the spinal canal. The following is a case report of such a tumour arising in the extradural space and causing spinal cord compression. Case report A 64-year-old retired schoolteacher was admitted to the Regional Neurological Centre in January 1974 with a I-month history of lower thoracic back pain. It was constant and radiated to the right hypochondrium. Three weeks before admission he had noticed that he was unsteady on walking and
Fig. 2. Photomicrograph illustrating marked vascularity of the edge of the lesion. Arrows point to vascular spaces. HE. ( x 160.)
that his legs had become weak. Shortly afterwards he developed hesitancy and urgency of micturition. Two days before admission he was unable to walk. For the past 8 years he had had hypertension and 2 years previously had had a cerebrovascular accident causing a left hemiparesis. On examination the patient had a localized tenderness over the spine of D9. Marked weakness was present in both lower limbs involving all muscle groups. There was no wasting, the tone was increased with brisk reflexes and the plantar responses were extensor. Pain and temperature sensation below the level of D9 were impaired. Both position and vibration sensations were absent below the hip joint and Romberg’s sign was positive. He was hypertensive (220/130 mm Hg) with retinal vascular changes and left ventricular voltage changes o n the ECG. Plain X-rays of the spine were normal. Myelography showed a complete block a t D9 with features suggesting a n extradural tumour. The cerebrospinal fluid protein was 76 mg per cent. A t operation the laminae from D8 to D11 were removed. A firm lobdated tumour was found in the extradural space pushing the cord anteriorly. The tumour was not attached to the dura or any nerve root. It was only loosely attached to Fig. 1. Photomicrograph illustrating a haphazard cellular arrangement with reticulin fibres outlining cells and demarcating vascular spaces. Reticulin. ( x 120.)
* Department of Neurosurgery and Neuropathology, Regional Neurological Centre, Newcastle General Hospital, Newcastle upon Tyne. 573
R. P. Sengupta et al.
Fig. 3. Photomicrograph showing vascular clefts and intercellular spaces, many of which contain red blood cells. HE. ( X 160.) fibrofatty tissue and required little dissection for complete removal. The patient made an uneventful recovery from the operation. Over a period of 4 weeks the power in the lower limbs returned to almost normal. The sensory deficit also recovered to a large extent and at the time of his discharge 4 weeks later he was able to walk unaided. Histological examination revealed a circumscribed cellular nodule measuring 3.8 x 2.5 x 1.5 cm attached a t one point to vascular fibrous fatty tissue. An incomplete fibrous capsule partially defined the edge of the nodule. Microscopic examination revealed the predominant cell type to be fibroblastic with vesicular lightly staining nuclei and eosinophilic cytoplasm. In the central region the cells were either plump and ovoid with an ill-defined eosinophilic cytoplasm and irregular reticulin fibres (Fig. I), or spindle-shaped with a compact fasciculated pattern and a regimented arrangement of reticulin fibres. In the peripheral regions the cells were less compact. Only occasional mitotic figures were identified and none had abnormal features. The stroma was highly vascular, particularly at the periphery (Fig. 2), with many vascular clefts, and spaces separated the fibroblastic cells. Throughout the tumour red blood cells lay in endothelialized clefts and non-endothelialized intercellular spaces (Fig. 3). Occasional vessels were cuffed with sparse accumulations of lymphoid cells. A small amount of collagen was present; no multinucleated cells were identified. Apart from the fat cells at the edge of the nodule no lipidcontaining cells were identified to suggest that the tumour had a lipomatous origin and the nodule had no features distinctive of a neurilemoma (no Antoni tissue type A and B and palisading).
Discussion The term ‘nodular fasciitis’ is now generally accepted as an apt description of a peculiar lesion which is 574
usually found in subcutaneous or deep fascia1 planes (Leading Article, 1973). The nodule is generally 1-3 cm in size and has distinctive histological features. It appears as a vascular proliferation of fibroblasts with a patchy lymphocytic infiltration, occasional islands of collagen and occasional giant or multinucleated cells (Price et al., 1961; Mackenzie, 1970). The cellularity of the lesion and the presence of mitotic figures have led to it being confused with a variety of mesodermal tumours and reactive processes, including sarcomas. It usually grows rapidly but it does not metastasize or recur if completelyexcised (Stout, 1961; Hutter et al., 1962). Although known to occur in a variety of locations. including the orbit (Font and Zimmerman, 1966), it has not to our knowledge been described as an extra, dural tumour causing compression of the spinal cordAnalysis of spinal cord tumours in the past (Elsberg, 1941; Kernohan, 1941; Austin, 1961) has listed ‘fibromata’ as constituting less than 1 per cent of extradural neoplasms. Unfortunately the histology of these fibromata was not described. The origin of nodular fasciitis is still in doubt. Traumatic stimulus has been cited as a possible aetiological factor (Soule, 1962), but in the present case there was no history of trauma to the back. The unusual situation in this case raises the questions of dermal implantation (Manno et al., 1962; James and Lassman, 1972), but a lumbar puncture had not been performed previously. In subcutaneous tissues nodular fasciitis is relatively harmless, but in this case in the confines of the vertebral canal it caused spinal cord compression and paraplegia. It is important to identify the lesion histologically because, despite its appearance, it is benign (Price et al., 1961; Hutter et al., 1962; Soule, 1962), and in the present case its removal at an early stage has prevented a permanent neurological deficit.
Acknowledgements The authors wish to thank Professor John Hankinson under whom this patient was admitted, and Professor B. E. Tomlinson for verifying the histology of the tumour. References (1961) The Spinal Cord. Springfield, 111. Thomas, pp. 104-105. ELSBERG c. A. (1941) Surgical Diseases of the Spinal Cord, Membranes and Nerve roots. New York, Hoeber, p. 49. FONT R . L. and ZIMMERMAN L. E. (1966) Nodular fasciitis of the eye and adnexa. Arch. Ophthalmol. 75, 475-48 1. HUTTER R. V. P., STEWART F. W. and FOOTE F. W. (1962) Fasciitis. Cancer 15, 992-1003. JAMES c. c. M. and LASSMAN L. P. (1972) Spinal Dysraphism. London, Butterworths, pp. 18-24, 57-60. KERNOHAN J. w. (1941) Tumours of the spinal cord. Arch. Pathol. 32, 843-883. AUSTIN G.
Nodular fasciitis LEADING ARTICLE
(1973) Nodular fasciitis. Lancet 1,
870. (1970) The Diflerential Diagnosis of Fibroblastic Disorders. Oxford, Blackwell, pp. 18-27. MANNO N. J., KIHLEIN A. and KERNOHAN J. w. (1962) Jntraspinal epidermoids. J. Neurosurg. 19, 754765. MACKENZIE D. H.
PRICE E. B. jun., SILLIPHANT w. M. and SHUMAN R.
(1961) Nodular fasciitis : a clinico-pathological analysis of 65 cases. Am. J. Clin. Pathol. 35, 122-136. SOULE E. G. ( I 962) Proliferative (nodular) fasciitis. Arch. Pathol. 73, 4 3 7 4 . STOUT A. P. (1961) Pseudosarcomatous fasciitis in children. Cancer 14, 1216-1222.
Errata British Journal of Surgery, Vol. 62: No. 3 : March 1975, p. 195. The legends to Figs. 2, 4 and 5 should read as follows: Fig. 2. Photomicrograph of regenerating liver at 72 hours . . . . Fig. 4. Case 7. Rise in serum bilirubin level after hepatic resection with unconjugated fraction as the main component (type I). Fig. 5. Case 6. Rise in serum bilirubin level after hepatic resection with the conjugated fraction as the main component (type 11).
575
