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Dermatofibrosarcoma Protuberans: A Tumor With Perineural and Endoneural Cell Features

Dermatofibrosarcoma Protuberans: A Tumor With Perineural and Endoneural Cell Features Abstract Seven new cases of dermatofibrosarcoma protuberans were studied. Most tumors were asymptomatic, multinodular, protuberant masses; five were on the trunk or abdomen and two on a thigh. Clinical diagnoses included dermatofibrosarcoma protuberans, dermatofibroma, metastasis, and lymphoma. Histologic examination showed a cartwheel or storiform pattern in each lesion; short fascicles of spindle-shaped tumor cells coursed in various directions and intersected at different angles. Melanocytes were present deep in one lesion. Electron microscopically, the tumor cells showed extensive cytoplasmic processes, moderately dense cytoplasmic matrix, elaborately convoluted nuclei, few profiles of rough-surfaced endoplasmic reticulum and Golgi lamellae, spotty basal laminalike material, and fibrous long-spacing collagen; conspicuously absent were intracellular lysosomes and lipid, and prominent cytoplasmic filamentation. The findings are sufficiently distinctive to be of diagnostic utility and suggest origin from a modified neural supporting cell, such as the endoneural or perineural cell. References 1. Darier J, Ferrand M: Dermatofibromes progressifs et récidivants ou fibrosarcomes de la peau . Ann Derm Syph 5:545-562, 1924. 2. Hoffmann E: Über das knollentreibende Fibrosarkom der Haut (Dermatofibrosarkoma protuberans) . Derm Ztschr 43:1-28, 1925.Crossref 3. Taylor HB, Helwig EB: Dermatofibrosarcoma protuberans: A study of 115 cases . Cancer 15:717-725, 1962.Crossref 4. Burkhardt BR, et al: Dermatofibrosarcoma protuberans: Study of 56 cases . Am J Surg 111:638-644, 1966.Crossref 5. McPeak CJ, Cruz T, Nicastri AD: Dermatofibrosarcoma protuberans: An analysis of 86 cases—five with metastasis . Ann Surg 166:803-816, 1967.Crossref 6. Smith JL Jr: Tumors of the Corium , in Helwig EB, Mostofi FK, (eds): The Skin . Baltimore, Williams & Wilkins Co, 1971, pp 533-577. 7. Fujita H, et al: Studies of mesodermal tumors: II. Dermatofibrosarcoma protuberans . Acta Dermatol (Kyoto) 57:4-6, 1962. 8. Hamamatsu T, Morohashi M, Suzuki K: Dermatofibrosarcoma protuberans . Rinsho Dermatol 10:798-811, 1968. 9. Fisher ER, Vuzevski VD: Cytogenesis of schwannoma (neurilemoma), neurofibroma, dermatofibroma, and dermatofibrosarcoma as revealed by electron microscopy . Am J Clin Pathol 49:141-154, 1968. 10. Tremblay M, Bonenfant JL, Cliche J: Le dermatofibrosarcome protubérant: Étude clinicopathologique de trente cas avec l'ultrastructure de deux cas . Union Med Can 99:871-876, 1970. 11. Stout AP, Lattes R: Tumors of the Soft Tissues . Washington, DC, Armed Forces Institute of Pathology, 1967, second series, pt (1) ,1967, p 44. 12. Bednář B: Storiform neurofibromas of the skin, pigmented and nonpigmented . Cancer 10:368-376, 1957.Crossref 13. Bednář B: Storiform neurofibroma in core of naevocellular naevi . J Pathol 101:199-201, 1970.Crossref 14. Hashimoto K, Pritzker M: Electron microscopic study of reticulohistiocytoma: An unusual case of congenital self-healing reticulohistiocytosis . Arch Dermatol 107:263-270, 1973.Crossref 15. Mihatsch-Konz B, Schaumburg-Lever G, Lever WF: Ultrastructure of dermatofibroma . Arch Derm Forsch 246:181-192, 1973.Crossref 16. Jakobiec FA, Tannenbaum M: The ultrastructure of orbital fibrosarcoma . Am J Ophthalmol 77:899-918, 1974. 17. Hashimoto K, Tarnowski W: Some new aspects of Langerhan's cell . Arch Dermatol 97:450-464, 1968.Crossref 18. Gianotti F, Caputo R: Skin ultrastructure in Hand-Schüller-Christian disease . Arch Dermatol 100:342-349, 1969.Crossref 19. Pineda A: Collagen formation by principal cells of acoustic tumors . Neurology 15:536-547, 1965.Crossref 20. Pineda A: Electronmicroscopy of the tumor cells in "neurofibromas" . J Neuropathol Exp Neurol 25:158-159, 1966. 21. Waggener I: Ultrastructure of benign peripheral nerve sheath tumors . Cancer 19:699-709, 1966.Crossref 22. Jakobiec FA, Jones IS: Neurogenic tumors of the orbit , in Jones IS, Jakobiec FA (eds): Diseases of the Orbit . New York, Harper & Row Publishers Inc, to be published. 23. Cravioto H, Lockwood R: Long spacing fibrous collagen in human acoustic nerve tumors . Cancer 23:891-899, 1969. 24. Iwamoto T, DeVoe AG: Electron microscopic studies on Fuch's combined dystrophy: I. Posterior portion of the cornea . Invest Ophthalmol 10:9-28, 1971. 25. Hashimoto K, Ohyama H: Cross-banded filamentous aggregation in the human dermis . J Invest Dermatol 62:106-112, 1974.Crossref 26. Banfield W, Lee CK, Lee CW: Myocardial collagen of the fibrous long-spacing type . Arch Pathol 95:262-266, 1973. 27. Gross J, Highberger H, Schmitt FO: Collagen structures considered as states of aggregation of a kinetic unit; the tropocollagen unit . Proc Natl Acad Sci USA 40:679-688, 1954.Crossref 28. Ferrans V, Roberts W: Structural features of cardiac myxomas: Histology, histochemistry, and electron microscopy . Human Pathol 4:111-146, 1973.Crossref 29. Church RL, Tanzer M, Pheiffer SE: Collagen and procollagen production by a clonal line of Schwann cells . Proc Natl Acad Sci USA 70:1943-1946, 1973.Crossref 30. Ross M, Reith E: Perineurium—Evidence for contractile elements . Science 165:604-605, 1969.Crossref 31. Olsson Y, Reese TS: Permeability of vasa nervorum and perineurium in mouse sciatic nerve studied by fluorescence and electron microscopy . J Neuropathol Exp Neurol 38:105-119, 1971.Crossref 32. Harkin J, Reed R: Tumors of the Peripheral Nervous System . Washington, DC, Armed Forces Institute of Pathology, 1969, Second series, pt (3) , pp 1-65. 33. Causey G: The Cell of Schwann . Edinburgh, LS Livingston, 1960, p 26. 34. Johnston M, Hazelton R: Embryonic origins of facial structures related to oral sensory and motor function , in Bosma JB (ed): Oral Sensory Perception: The Mouth of the Infant . Springfield, Ill, Charles C Thomas Publisher, 1972, pp 76-97. 35. Johnston M, Listgarten M: The migration, interaction and early differentiation of oro-facial tissues , in Slavkin HS, Bavetta L (eds): Developmental Aspects of Oral Biology . New York, Academic Press, 1972, 55-80. 36. Hogan M, Alvarado J, Weddell J: Histology of the Human Eye . Philadelphia, WB Saunders Co, 1971, pp 86-93 37. 154-165. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Dermatofibrosarcoma Protuberans: A Tumor With Perineural and Endoneural Cell Features

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Publisher
American Medical Association
Copyright
Copyright © 1974 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1974.01630120024006
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Abstract

Abstract Seven new cases of dermatofibrosarcoma protuberans were studied. Most tumors were asymptomatic, multinodular, protuberant masses; five were on the trunk or abdomen and two on a thigh. Clinical diagnoses included dermatofibrosarcoma protuberans, dermatofibroma, metastasis, and lymphoma. Histologic examination showed a cartwheel or storiform pattern in each lesion; short fascicles of spindle-shaped tumor cells coursed in various directions and intersected at different angles. Melanocytes were present deep in one lesion. Electron microscopically, the tumor cells showed extensive cytoplasmic processes, moderately dense cytoplasmic matrix, elaborately convoluted nuclei, few profiles of rough-surfaced endoplasmic reticulum and Golgi lamellae, spotty basal laminalike material, and fibrous long-spacing collagen; conspicuously absent were intracellular lysosomes and lipid, and prominent cytoplasmic filamentation. The findings are sufficiently distinctive to be of diagnostic utility and suggest origin from a modified neural supporting cell, such as the endoneural or perineural cell. References 1. Darier J, Ferrand M: Dermatofibromes progressifs et récidivants ou fibrosarcomes de la peau . Ann Derm Syph 5:545-562, 1924. 2. Hoffmann E: Über das knollentreibende Fibrosarkom der Haut (Dermatofibrosarkoma protuberans) . Derm Ztschr 43:1-28, 1925.Crossref 3. Taylor HB, Helwig EB: Dermatofibrosarcoma protuberans: A study of 115 cases . Cancer 15:717-725, 1962.Crossref 4. Burkhardt BR, et al: Dermatofibrosarcoma protuberans: Study of 56 cases . Am J Surg 111:638-644, 1966.Crossref 5. McPeak CJ, Cruz T, Nicastri AD: Dermatofibrosarcoma protuberans: An analysis of 86 cases—five with metastasis . Ann Surg 166:803-816, 1967.Crossref 6. Smith JL Jr: Tumors of the Corium , in Helwig EB, Mostofi FK, (eds): The Skin . Baltimore, Williams & Wilkins Co, 1971, pp 533-577. 7. Fujita H, et al: Studies of mesodermal tumors: II. Dermatofibrosarcoma protuberans . Acta Dermatol (Kyoto) 57:4-6, 1962. 8. Hamamatsu T, Morohashi M, Suzuki K: Dermatofibrosarcoma protuberans . Rinsho Dermatol 10:798-811, 1968. 9. Fisher ER, Vuzevski VD: Cytogenesis of schwannoma (neurilemoma), neurofibroma, dermatofibroma, and dermatofibrosarcoma as revealed by electron microscopy . Am J Clin Pathol 49:141-154, 1968. 10. Tremblay M, Bonenfant JL, Cliche J: Le dermatofibrosarcome protubérant: Étude clinicopathologique de trente cas avec l'ultrastructure de deux cas . Union Med Can 99:871-876, 1970. 11. Stout AP, Lattes R: Tumors of the Soft Tissues . Washington, DC, Armed Forces Institute of Pathology, 1967, second series, pt (1) ,1967, p 44. 12. Bednář B: Storiform neurofibromas of the skin, pigmented and nonpigmented . Cancer 10:368-376, 1957.Crossref 13. Bednář B: Storiform neurofibroma in core of naevocellular naevi . J Pathol 101:199-201, 1970.Crossref 14. Hashimoto K, Pritzker M: Electron microscopic study of reticulohistiocytoma: An unusual case of congenital self-healing reticulohistiocytosis . Arch Dermatol 107:263-270, 1973.Crossref 15. Mihatsch-Konz B, Schaumburg-Lever G, Lever WF: Ultrastructure of dermatofibroma . Arch Derm Forsch 246:181-192, 1973.Crossref 16. Jakobiec FA, Tannenbaum M: The ultrastructure of orbital fibrosarcoma . Am J Ophthalmol 77:899-918, 1974. 17. Hashimoto K, Tarnowski W: Some new aspects of Langerhan's cell . Arch Dermatol 97:450-464, 1968.Crossref 18. Gianotti F, Caputo R: Skin ultrastructure in Hand-Schüller-Christian disease . Arch Dermatol 100:342-349, 1969.Crossref 19. Pineda A: Collagen formation by principal cells of acoustic tumors . Neurology 15:536-547, 1965.Crossref 20. Pineda A: Electronmicroscopy of the tumor cells in "neurofibromas" . J Neuropathol Exp Neurol 25:158-159, 1966. 21. Waggener I: Ultrastructure of benign peripheral nerve sheath tumors . Cancer 19:699-709, 1966.Crossref 22. Jakobiec FA, Jones IS: Neurogenic tumors of the orbit , in Jones IS, Jakobiec FA (eds): Diseases of the Orbit . New York, Harper & Row Publishers Inc, to be published. 23. Cravioto H, Lockwood R: Long spacing fibrous collagen in human acoustic nerve tumors . Cancer 23:891-899, 1969. 24. Iwamoto T, DeVoe AG: Electron microscopic studies on Fuch's combined dystrophy: I. Posterior portion of the cornea . Invest Ophthalmol 10:9-28, 1971. 25. Hashimoto K, Ohyama H: Cross-banded filamentous aggregation in the human dermis . J Invest Dermatol 62:106-112, 1974.Crossref 26. Banfield W, Lee CK, Lee CW: Myocardial collagen of the fibrous long-spacing type . Arch Pathol 95:262-266, 1973. 27. Gross J, Highberger H, Schmitt FO: Collagen structures considered as states of aggregation of a kinetic unit; the tropocollagen unit . Proc Natl Acad Sci USA 40:679-688, 1954.Crossref 28. Ferrans V, Roberts W: Structural features of cardiac myxomas: Histology, histochemistry, and electron microscopy . Human Pathol 4:111-146, 1973.Crossref 29. Church RL, Tanzer M, Pheiffer SE: Collagen and procollagen production by a clonal line of Schwann cells . Proc Natl Acad Sci USA 70:1943-1946, 1973.Crossref 30. Ross M, Reith E: Perineurium—Evidence for contractile elements . Science 165:604-605, 1969.Crossref 31. Olsson Y, Reese TS: Permeability of vasa nervorum and perineurium in mouse sciatic nerve studied by fluorescence and electron microscopy . J Neuropathol Exp Neurol 38:105-119, 1971.Crossref 32. Harkin J, Reed R: Tumors of the Peripheral Nervous System . Washington, DC, Armed Forces Institute of Pathology, 1969, Second series, pt (3) , pp 1-65. 33. Causey G: The Cell of Schwann . Edinburgh, LS Livingston, 1960, p 26. 34. Johnston M, Hazelton R: Embryonic origins of facial structures related to oral sensory and motor function , in Bosma JB (ed): Oral Sensory Perception: The Mouth of the Infant . Springfield, Ill, Charles C Thomas Publisher, 1972, pp 76-97. 35. Johnston M, Listgarten M: The migration, interaction and early differentiation of oro-facial tissues , in Slavkin HS, Bavetta L (eds): Developmental Aspects of Oral Biology . New York, Academic Press, 1972, 55-80. 36. Hogan M, Alvarado J, Weddell J: Histology of the Human Eye . Philadelphia, WB Saunders Co, 1971, pp 86-93 37. 154-165.

Journal

Archives of DermatologyAmerican Medical Association

Published: Dec 1, 1974

References