- Email: [email protected]
Primary leiomyosarcoma of the skin
Journal of the American Academy of Dermatology
Roth et al.
18. 19. 20. 21. 22. 23.
abetic patients resolved by treatment with human insulin. Ann Intern Med 1985;102:790-1. Traisman HS, Traisman AS, Rice H1V[.Lipodystrophy due to injections. AJDC 1968;115:392. Wilson AA, Sissons JGP, Morgan OS. Multipte autoimmune diseases with bilateral optic atrophy and lipodystrophy, Ann Intern Med 1978;89:72-3. Winkelmann R_K,Padilha-Goncalves A. Connective tissue panniculitis. Arch Dermatol 1980;116:291-3. Billings JK, Milgraum SS; Gupta AK, et al. Lipoatrophic Panniculitis: a Possible autoimmune inflammatory disease of fat. Arch Dermatol 1987;123:1662-6. Solom0nAR, KantakAG, Ramierez JE, etal. Suppressorcytotoxic T-lymphocyte panniculitis. Pediatr Dermatol 1986;3:295-9. Alarcon-Segoria D, Ramos-Niembro F. Association of partial lipodystrophy and Sj6gren's syndrome. Ann Intern Med 1976;85:474-5.
24. [pp MM, Howard N J, Tervo R.C, et al. Sicca syndrome and total lipodystrophy:a case in a 15-year-old female patient. Ann Intern Med 1976;85:443-6. 25. Peters MS, Winkelmann RK. Localized lipoatrophy (atrophic connectivetissuediseasepanniculitis). Arch Dermatol
1980;116:1363-8. 26. Shelley WB, Izumi AK. Annular atrophy of the an/des: a case of partial lipodystrophy. Arch Dermatol 1970;102: 326-9. 27. JablonskaS, Szczepanski A, Gorkiew!czA. Lipoatrophyof the ankles and its relation to other lipoatrophies. Acta Derm Venereol (Stockh) 1975;55:135-40. 28. Gupta AK~Rasmussen JE. Multiple areas of lecalized tissue loss in a child. Arch Dermatol 1986;122:!199-204. 29. Nelson HM. Atrophic annular panniculitis of the ankles. Clin Exp Dermatol 1988;13":111-3.
Primary leiomyosarcoma of the skin ?
Case report and review of the literature Lori L. Davidson, MD, Marc L. Frost, MD, C. William Hanke, MD, and Warren W. Epinette, M D Indianapolis, Indiana Leiomyosarcoma is a rare tumor usual/y treated by wide local excision. We present a case of primary leiomyosarcoma of the skin on the anterior aspect of the chest wall treated by Mohs micrographic surgery, This is only the second-reported case so treated. The patient has remained recurrence-free for 30 months. (J AM ACAD DERMATOL1989;21:1156-60.) Primary leiomyosarcoma of the skin or subcutaneous tissue is rare. A recent review revealed only 106 cases, t-4 Although subcutaneous leiomyosarcoma m a y metastasize, cutaneous leiomyosarcoma is biologically less aggressive and is more likely to recur locally. Surgical excision with wide margins has been recommended for cutaneous leiomyosarcoma), 2, 5 W e report a patient with primary cutaneous leiomyosarcoma treated with Mohs micrograPhic surgery who has remained recurrence free for 30 months.
CASE REPORT A 48-year-old white man came to the Indiana University Department of Dermatology in May 1985 for examFrom the Department of Dermatology,Indiana UniversityMedical Center. No reprintsavailable.
1156
ination of a slightly tender nodule overlying the sternal area. The nodule had been slowly enlarging for I year. There was no history of previous trauma. The patierit's general health was excellent. Physical examination revealed a firm, slightly tender, poorly circumscribed 1.0 × 1.0 cm dermal nodule Overlying the inferior aspect of the sternum. The epidermis was Clinically uninvolved. The nodule was adherent to the dermis but n0tto underlying structuresl NO lymphadenopathy was present. The nodule was excised and submitred for histopathologic examination.
Hist0pathologic study Examination of the lesion revealed in the reticular dermis a tumor consisting of cigar-shaped spindle Cells with hyperchromatic nuclei. The tumor was arranged into irregular fascicles that infiltrated the .space between the collagen bundles (Figs. 1 and 2). Occasional large atypical nuclei and mitotic figures were present (Fig. 3). The tumor extended to the lateral
Volume 21 Number 5, Part 2 November 1989
Primary leiomyosarcoma
Fig. 1. The hyperceUular dermal spindle cell tumor arranged in fascicles and bundles. (Hematoxylin-eosin stain; x 100.)
Fig. 2. Cigar-shaped spindle cells with hyperchromatic nuclei and mitotic figures are present. (×450.)
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Journal of the American Academy of Dermatology
Davidson et aL
Fig. 3. Tumor has fascicular arrangement. Numerous mitoses are present. (×450.)
and deep margins of the specimen but not into the subcutaneous fat (Fig. 4). The overlying epidermis was normal. Masson's trichrome stained the spindle cells red, confirming their smooth muscle origin. Stains for S-100 protein were negative.
Treatment With local anesthesic, Mohs micrographic surgery was performed to excise the residt~al leiomyosarcoma. After two stages of Mohs micrographic surgery with the use of 16 horizontal frozen sections, a tumor-free plan e was achieved. The final surgical defect was 4.5 × 3.0 cm. The wound was allowed to heal without closure to facilitate observances of a tumor recurrence. Follow-up at 30 months revealed a well-healed chest scar without evidence of local recurrence. A chest roentgenogram was normal.
DISCUSSION Leiomyosarcomas usually occur in the fifth to seventh decades of life and are most common in white persons. 2, 6 A review of 80 cases by Fields and Helwig 2 showed a 3:1 male/female ratio. Generally the tumor appears as a firm, solitary nodule. The majority of superficial leiomyosarcomas occur on the lower extremities. They may be either tender or asymptomatic. Subcutaneous leiomyosarcomas are vascular in origin and arise from the muscular media of blood vessels.7 Because of the lesions' rapid rate of growth and their tendency to metastasize, patients with these tumors have a poor prognosis. 7 Histologically, subcutaneous leiomyosarcomas are different from cutaneous leiomyosarcomas in that the former are
Volume 21 Number 5, Part 2 November 1989
Primary leiomyosarcoma 1159
Fig. 4. Tumor extends to lateral and deep margins of specimen but not into subcutaneous fat. (X20.)
well circumscribed and have thin-walled blood vessels of various shapes and sizes within them. 2,7 Patients with cutaneous or intradermal leiomyosarcomas have a better prognosis] These tumors tend to recur locally rather than to metastasize. They are usually much smaller than subcutaneous leiomyosarcomas when first detected and diagnosed2, 6 and are more likely to produce such changes in the overlying epidermis as discoloration, umbilication, and ulceration. 2, 6 Cutaneous leiomyosarcomas originate in the arrector muscle of the hair follicle or in the smooth muscle surrounding sweat glands. 3 Histologically, intradermal leiomyosarcomas are composed of ill-defined, poorly to moderately differentiated spindle cells, which are haphazardly arranged in dense, interlacing fascicles that may extend between collagen bundles. 7 Cutaneous leiomyosarcomas show a preference for the hair-bearing extensor surfaces. 2' 6 In our patient the tumor was located on the anterior aspect & t h e chest. Fields and Helwig 2 reported only 6.1% of 65 cutaneous tumors in this location. Local recurrences developed in 32% of the leiomyosarcomas that were confined to the dermis, compared with 47% of those arising in the subcutis. 2 Recurrences usually occurred within 1 to 5 years of the initial surgery. 2 In contrast to the 33% incidence of metastases in subcutaneous leiomyosarcomas, metastatic disease in cutaneous leiomyosarcoma is rarely described, and the diagnosis in these cases has been ques-
tioned. 2 Treatment for leiomyosarcomas of the skin has consisted primarily of wide excision but has also included amputation, fulguration, and radiotherapy. 2 Tumors treated with radiotherapy required subsequent surgical intervention for local recurrence. 2 It is believed that only surgical treatment can provide a cure. Currently the most effective treatment is believed to be wide local excision with a 3 to 5 cm margin (including the subcutaneous tissue and fascia) with the subsequent placement of a sprit-thickness skin graft. 1,2, 5 In our case the patient was successfully treated without the removal of substantial tissue with Mohs micrographic surgery. Because most recurrences of leiomyosarcomas of the skin occur within 5 years of the initial surgery, s our patient's condition without recurrence after 30 months is encouraging. Mohs micrographic surgery offers the advantages of reliability and conservatism in the treatment of soft tissue sarcomas that are known for their tendency to develop clinically undetectable extensions from the main tumor mass. 8 Mohs surgery has been used for removal of other sarcomas such as dermatofibroma protuberans, fibrosarcoma, neurofibrosarcoma, angiofibrosarcoma, hemangiosarcoma, lymphangiosarcoma, myosarcoma, reticulum cell sarcoma, rhabdomyosarcoma, synoviosarcoma, lymphosareoma, liposarcoma, neurosarcoma, fibromyxosarcoma, and fibroxanthosarcoma. 8, 9 The use of Mohs micrographic surgery in the
1160
Davidson et aL
m a n a g e m e n t o f l e i o m y o s a r c o m a h a s been m e n tioned only o n c e briefly in a series of genital tumors.l° A 36-year-old m a n w i t h l e i o m y o s a r c o m a o f the penis was t r e a t e d with M o h s m i c r o g r a p h i c surgery a n d r e m a i n e d t u m o r - f r e e a f t e r 18 months, F u r t h e r clinical or histologic details w e r e not reported. 1° REFERENCES
1. Aranha GV, Molnar ZV, Reyes CV, et al. Leiomyosarcoma of the skin: a case report. J Surg Oncol 1982;19:87-9. 2. Fields JP, Hdwig EB. Leiomyosarcoma of the skin and subcutaneous tissue. Cancer 1981;47:156-69. 3. Jegasothy BV, Gilgor RS, Hail DM. Leiomyosarcoma of the skin and subcutaneous tissue. Arch Dermatol 1981; 117:478-8 I. 4. Oreliana-Diaz O, Hernandez-Perez E. Leiomyoma curls and leiomyosarcoma: a 10-year study and a short review. J Dermatol Surg Oncol 1983;9:283-7.
Journal of the American Academy of Dermatology
5. Chaves E, Sa HH, Gadelha N, et al. Leiomyosarcoma in the skin. Acta Derm Venereol (Stookh) 1972;52:288-90. 6. Enzinger FM, Weiss SW. Soft tissue tumors. St Louis: CV Mosby, 1983;309-11. 7. Lever WF, ed. Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983;305-6. 8. Mobs FE. Chemosurgery. Springfield, II1:Charles C Tho~ mas, 1978;249-55. 9. Hess KA, Hanke CW, Estes NC, et al. Chemosurgical reports: myxoid dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 1985;11:268-71. 10. Brown MD, Zachary CB, Grekin RC, et al. Genital tumors: their management by micrographic surgery. J AM ACAD DERMATOL1988;18:115-22. 11. Panabokke RG, Attygalle LS. Leiomyosarooma of the skin [Letter]. Br J Dermatol 1967;79:305-6. 12. Rising JA, Booth E. Primary leiomyosarcoma of the skin with lymphatic spread. Areh Pathol 1966;81:94-6. 13. Stout AP, Hill WT. Leiomyosarcoma of the superficial soft tissue. Cancer 1958; 11:844-54.
Roth et al.
18. 19. 20. 21. 22. 23.
abetic patients resolved by treatment with human insulin. Ann Intern Med 1985;102:790-1. Traisman HS, Traisman AS, Rice H1V[.Lipodystrophy due to injections. AJDC 1968;115:392. Wilson AA, Sissons JGP, Morgan OS. Multipte autoimmune diseases with bilateral optic atrophy and lipodystrophy, Ann Intern Med 1978;89:72-3. Winkelmann R_K,Padilha-Goncalves A. Connective tissue panniculitis. Arch Dermatol 1980;116:291-3. Billings JK, Milgraum SS; Gupta AK, et al. Lipoatrophic Panniculitis: a Possible autoimmune inflammatory disease of fat. Arch Dermatol 1987;123:1662-6. Solom0nAR, KantakAG, Ramierez JE, etal. Suppressorcytotoxic T-lymphocyte panniculitis. Pediatr Dermatol 1986;3:295-9. Alarcon-Segoria D, Ramos-Niembro F. Association of partial lipodystrophy and Sj6gren's syndrome. Ann Intern Med 1976;85:474-5.
24. [pp MM, Howard N J, Tervo R.C, et al. Sicca syndrome and total lipodystrophy:a case in a 15-year-old female patient. Ann Intern Med 1976;85:443-6. 25. Peters MS, Winkelmann RK. Localized lipoatrophy (atrophic connectivetissuediseasepanniculitis). Arch Dermatol
1980;116:1363-8. 26. Shelley WB, Izumi AK. Annular atrophy of the an/des: a case of partial lipodystrophy. Arch Dermatol 1970;102: 326-9. 27. JablonskaS, Szczepanski A, Gorkiew!czA. Lipoatrophyof the ankles and its relation to other lipoatrophies. Acta Derm Venereol (Stockh) 1975;55:135-40. 28. Gupta AK~Rasmussen JE. Multiple areas of lecalized tissue loss in a child. Arch Dermatol 1986;122:!199-204. 29. Nelson HM. Atrophic annular panniculitis of the ankles. Clin Exp Dermatol 1988;13":111-3.
Primary leiomyosarcoma of the skin ?
Case report and review of the literature Lori L. Davidson, MD, Marc L. Frost, MD, C. William Hanke, MD, and Warren W. Epinette, M D Indianapolis, Indiana Leiomyosarcoma is a rare tumor usual/y treated by wide local excision. We present a case of primary leiomyosarcoma of the skin on the anterior aspect of the chest wall treated by Mohs micrographic surgery, This is only the second-reported case so treated. The patient has remained recurrence-free for 30 months. (J AM ACAD DERMATOL1989;21:1156-60.) Primary leiomyosarcoma of the skin or subcutaneous tissue is rare. A recent review revealed only 106 cases, t-4 Although subcutaneous leiomyosarcoma m a y metastasize, cutaneous leiomyosarcoma is biologically less aggressive and is more likely to recur locally. Surgical excision with wide margins has been recommended for cutaneous leiomyosarcoma), 2, 5 W e report a patient with primary cutaneous leiomyosarcoma treated with Mohs micrograPhic surgery who has remained recurrence free for 30 months.
CASE REPORT A 48-year-old white man came to the Indiana University Department of Dermatology in May 1985 for examFrom the Department of Dermatology,Indiana UniversityMedical Center. No reprintsavailable.
1156
ination of a slightly tender nodule overlying the sternal area. The nodule had been slowly enlarging for I year. There was no history of previous trauma. The patierit's general health was excellent. Physical examination revealed a firm, slightly tender, poorly circumscribed 1.0 × 1.0 cm dermal nodule Overlying the inferior aspect of the sternum. The epidermis was Clinically uninvolved. The nodule was adherent to the dermis but n0tto underlying structuresl NO lymphadenopathy was present. The nodule was excised and submitred for histopathologic examination.
Hist0pathologic study Examination of the lesion revealed in the reticular dermis a tumor consisting of cigar-shaped spindle Cells with hyperchromatic nuclei. The tumor was arranged into irregular fascicles that infiltrated the .space between the collagen bundles (Figs. 1 and 2). Occasional large atypical nuclei and mitotic figures were present (Fig. 3). The tumor extended to the lateral
Volume 21 Number 5, Part 2 November 1989
Primary leiomyosarcoma
Fig. 1. The hyperceUular dermal spindle cell tumor arranged in fascicles and bundles. (Hematoxylin-eosin stain; x 100.)
Fig. 2. Cigar-shaped spindle cells with hyperchromatic nuclei and mitotic figures are present. (×450.)
1157
1158
Journal of the American Academy of Dermatology
Davidson et aL
Fig. 3. Tumor has fascicular arrangement. Numerous mitoses are present. (×450.)
and deep margins of the specimen but not into the subcutaneous fat (Fig. 4). The overlying epidermis was normal. Masson's trichrome stained the spindle cells red, confirming their smooth muscle origin. Stains for S-100 protein were negative.
Treatment With local anesthesic, Mohs micrographic surgery was performed to excise the residt~al leiomyosarcoma. After two stages of Mohs micrographic surgery with the use of 16 horizontal frozen sections, a tumor-free plan e was achieved. The final surgical defect was 4.5 × 3.0 cm. The wound was allowed to heal without closure to facilitate observances of a tumor recurrence. Follow-up at 30 months revealed a well-healed chest scar without evidence of local recurrence. A chest roentgenogram was normal.
DISCUSSION Leiomyosarcomas usually occur in the fifth to seventh decades of life and are most common in white persons. 2, 6 A review of 80 cases by Fields and Helwig 2 showed a 3:1 male/female ratio. Generally the tumor appears as a firm, solitary nodule. The majority of superficial leiomyosarcomas occur on the lower extremities. They may be either tender or asymptomatic. Subcutaneous leiomyosarcomas are vascular in origin and arise from the muscular media of blood vessels.7 Because of the lesions' rapid rate of growth and their tendency to metastasize, patients with these tumors have a poor prognosis. 7 Histologically, subcutaneous leiomyosarcomas are different from cutaneous leiomyosarcomas in that the former are
Volume 21 Number 5, Part 2 November 1989
Primary leiomyosarcoma 1159
Fig. 4. Tumor extends to lateral and deep margins of specimen but not into subcutaneous fat. (X20.)
well circumscribed and have thin-walled blood vessels of various shapes and sizes within them. 2,7 Patients with cutaneous or intradermal leiomyosarcomas have a better prognosis] These tumors tend to recur locally rather than to metastasize. They are usually much smaller than subcutaneous leiomyosarcomas when first detected and diagnosed2, 6 and are more likely to produce such changes in the overlying epidermis as discoloration, umbilication, and ulceration. 2, 6 Cutaneous leiomyosarcomas originate in the arrector muscle of the hair follicle or in the smooth muscle surrounding sweat glands. 3 Histologically, intradermal leiomyosarcomas are composed of ill-defined, poorly to moderately differentiated spindle cells, which are haphazardly arranged in dense, interlacing fascicles that may extend between collagen bundles. 7 Cutaneous leiomyosarcomas show a preference for the hair-bearing extensor surfaces. 2' 6 In our patient the tumor was located on the anterior aspect & t h e chest. Fields and Helwig 2 reported only 6.1% of 65 cutaneous tumors in this location. Local recurrences developed in 32% of the leiomyosarcomas that were confined to the dermis, compared with 47% of those arising in the subcutis. 2 Recurrences usually occurred within 1 to 5 years of the initial surgery. 2 In contrast to the 33% incidence of metastases in subcutaneous leiomyosarcomas, metastatic disease in cutaneous leiomyosarcoma is rarely described, and the diagnosis in these cases has been ques-
tioned. 2 Treatment for leiomyosarcomas of the skin has consisted primarily of wide excision but has also included amputation, fulguration, and radiotherapy. 2 Tumors treated with radiotherapy required subsequent surgical intervention for local recurrence. 2 It is believed that only surgical treatment can provide a cure. Currently the most effective treatment is believed to be wide local excision with a 3 to 5 cm margin (including the subcutaneous tissue and fascia) with the subsequent placement of a sprit-thickness skin graft. 1,2, 5 In our case the patient was successfully treated without the removal of substantial tissue with Mohs micrographic surgery. Because most recurrences of leiomyosarcomas of the skin occur within 5 years of the initial surgery, s our patient's condition without recurrence after 30 months is encouraging. Mohs micrographic surgery offers the advantages of reliability and conservatism in the treatment of soft tissue sarcomas that are known for their tendency to develop clinically undetectable extensions from the main tumor mass. 8 Mohs surgery has been used for removal of other sarcomas such as dermatofibroma protuberans, fibrosarcoma, neurofibrosarcoma, angiofibrosarcoma, hemangiosarcoma, lymphangiosarcoma, myosarcoma, reticulum cell sarcoma, rhabdomyosarcoma, synoviosarcoma, lymphosareoma, liposarcoma, neurosarcoma, fibromyxosarcoma, and fibroxanthosarcoma. 8, 9 The use of Mohs micrographic surgery in the
1160
Davidson et aL
m a n a g e m e n t o f l e i o m y o s a r c o m a h a s been m e n tioned only o n c e briefly in a series of genital tumors.l° A 36-year-old m a n w i t h l e i o m y o s a r c o m a o f the penis was t r e a t e d with M o h s m i c r o g r a p h i c surgery a n d r e m a i n e d t u m o r - f r e e a f t e r 18 months, F u r t h e r clinical or histologic details w e r e not reported. 1° REFERENCES
1. Aranha GV, Molnar ZV, Reyes CV, et al. Leiomyosarcoma of the skin: a case report. J Surg Oncol 1982;19:87-9. 2. Fields JP, Hdwig EB. Leiomyosarcoma of the skin and subcutaneous tissue. Cancer 1981;47:156-69. 3. Jegasothy BV, Gilgor RS, Hail DM. Leiomyosarcoma of the skin and subcutaneous tissue. Arch Dermatol 1981; 117:478-8 I. 4. Oreliana-Diaz O, Hernandez-Perez E. Leiomyoma curls and leiomyosarcoma: a 10-year study and a short review. J Dermatol Surg Oncol 1983;9:283-7.
Journal of the American Academy of Dermatology
5. Chaves E, Sa HH, Gadelha N, et al. Leiomyosarcoma in the skin. Acta Derm Venereol (Stookh) 1972;52:288-90. 6. Enzinger FM, Weiss SW. Soft tissue tumors. St Louis: CV Mosby, 1983;309-11. 7. Lever WF, ed. Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983;305-6. 8. Mobs FE. Chemosurgery. Springfield, II1:Charles C Tho~ mas, 1978;249-55. 9. Hess KA, Hanke CW, Estes NC, et al. Chemosurgical reports: myxoid dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 1985;11:268-71. 10. Brown MD, Zachary CB, Grekin RC, et al. Genital tumors: their management by micrographic surgery. J AM ACAD DERMATOL1988;18:115-22. 11. Panabokke RG, Attygalle LS. Leiomyosarooma of the skin [Letter]. Br J Dermatol 1967;79:305-6. 12. Rising JA, Booth E. Primary leiomyosarcoma of the skin with lymphatic spread. Areh Pathol 1966;81:94-6. 13. Stout AP, Hill WT. Leiomyosarcoma of the superficial soft tissue. Cancer 1958; 11:844-54.