- Email: [email protected]
A case of extraskeletal osteosarcoma with metastasis to the skin
124 Case reports
may predispose to trauma, hence, resulting in tissue injury. First, the lesion on the ear is located at a point between a larger and smaller part of helix. This point of the ear probably sustains the largest shearing stress during gestation. Secondly, no subcutaneous tissue in the lateral ear insulates it from trauma. Thirdly, only small blood vessels supply nourishment to epidermis, dermis, and perichondrium. So even minor trauma to the ear may induce ischemic injury. This then may initiate the process of calcification in the tissue. For these reasons we think that calcinosis on the ear in the exact same location is not coincidental and should be a distinct group of calcinosis. We speculate that this form of calcinosis cutis is induced by trauma during gestation. The minor bending of the ear can cause enough trauma to result in calcification. In this way, congenital calcinosis cutis of the ear should be regarded as one type of dystrophic calcification.
J AM ACAD DERMATOL JULY 2003
REFERENCES 1. Winer LH. Solitary nodular calcification of the skin. Arch Dermatol Syph 1952;66:204-11. 2. Mehregan AH. Calcinosis cutis: a review of the clinical forms and report of 75 cases. Semin Dermatol 1984;3:53-61. 3. Younger B, Swan JW. Solitary congenital calcified nodule of the ear. Am J Dermatopathol 1982;4:377-80. 4. Mallory SB, Tryka AF. Solitary congenital nodule of the ear. Arch Dermatol 1988;124:769-74. 5. Lucky AW, Prendiville JS. Solitary congenital nodule on the ear of an infant. Pediatr Dermatol 1993;10:88-90. 6. Evans MJ, Blessing K, Gray ES. Subepidermal calcified nodule in children: a clinicopathologic study of 21 cases. Pediatr Dermatol 1995;12:307-10. 7. Sell EJ, Hansen RC, Struck-Pierce S. Calcified nodules on the heel: a complication of neonatal intensive care. J Pediatr 1980;96: 473-5. 8. Speer ME, Rudolph AJ. Calcification of superficial scalp veins secondary to intravenous infusion of sodium bicarbonate and calcium chloride. Cutis 1983;32:65-6. 9. Wiley HE, Eaglstein WE. Calcinosis cutis in children following electroencephalography. JAMA 1979;242:455-6. 10. Johnson RC, Fitzpatrick JE, Hahn DE. Calcinosis cutis following electromyographic examination. Cutis 1993;52:161-4.
A case of extraskeletal osteosarcoma with metastasis to the skin Seana P. Covello, MD, Tatyana R. Humphreys, MD, and Jason B. Lee, MD Philadelphia, Pennsylvania We present an 83-year-old woman with extraskeletal osteosarcoma (ESOS) of the breast who developed metastasis to the scalp. Skeletal osteosarcoma is the most common primary malignant neoplasm of the bone, predominantly occurring in the metaphysis of the long bones of adolescents and young adults. ESOS, in contrast, occurs primarily in the fifth and sixth decades of life, most commonly in the soft tissue of the thigh. Although the lung is overwhelmingly the most common site of metastasis for both skeletal osteosarcoma and ESOS, the skin is an uncommon metastatic site with only a few reported cases. Metastasis of ESOS to the skin is an exceedingly infrequent phenomenon, which may be a sign of widespread metastases foreboding a grim prognosis. (J Am Acad Dermatol 2003;49:124-7.)
O
steosarcoma is a malignant neoplasm of the bone in which the mesenchymal neoplastic cells have the ability to produce osteoid or immature bone. It is the most common primary malignant neoplasm of the bone, predominantly oc-
From the Department of Dermatology and Cutaneous Biology, Jefferson Medical College. Funding sources: None. Conflict of interest: None identified. Reprint requests: Jason B. Lee, MD, Jefferson Medical College, Department of Dermatology and Cutaneous Biology, 833 Chestnut St, Suite 740, Philadelphia, PA 19107. E-mail: jason.b.lee@ mail.tju.edu. Copyright © 2003 by the American Academy of Dermatology, Inc. 0190-9622/2003/$30.00 ⫹ 0 doi:10.1067/mjd.2003.297
curring in the metaphysis of the long bones of adolescents and young adults, corresponding to the peak period of skeletal growth.1 Before the advent of chemotherapy, specifically doxorubicin, highdose methotrexate, cisplatin, and ifosfamide,2 the 5-year survival was dismal at 20%, but now has risen to 63%.3 The lung is the most common site of metastasis4 with very few reported cases of metastasis to the skin.5-7 Rarely, osteosarcomas occur at sites other than the bone, the so-called extraskeletal osteosarcoma (ESOS). ESOS has been reported to occur in virtually every organ, including the skin.8,9 However, the most common site is the thigh, which usually presents as a deep-seated, soft-tissue mass.10 In contrast to skeletal osteosarcoma (SOS), ESOS is
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primarily observed in the fifth and sixth decades of life.11,12 ESOS may develop at sites of trauma (6%24%) and areas of radiation (4%-10%).10-14 ESOS has a 5-year survival that ranges from 25% to 37%.12-14 The lower survival outcome compared with SOS may be partly explained by the older age group. Radical wide excision affords the best chance for cure because of its high recurrence rate and early metastasis. Similar to SOS, the lung is overwhelmingly the most common site of metastasis, whereas metastasis to the skin appears to be exceedingly rare. There are 2 microscopic components of SOS and ESOS, namely sarcomatous neoplastic cells and extracellular matrix, which consist of osteoid and/or immature bone. Three main histopathologic subtypes are recognized depending on this matrix product: osteoblastic; chondroblastic; and fibroblastic.15 When osteoclast-like cells are abundant, these neoplasms are descriptively designated as giant-cell-rich or osteoclastic osteosarcomas. When bone formation is inconspicuous in the fibroblastic subtype, the histopathologic findings may be difficult to distinguish from malignant fibrous histiocytoma,10 but alkaline phosphatase staining on fresh tissue may be a helpful distinguishing marker, which stains ESOS prominently.12 Fibroblastic ESOS and malignant fibrous histiocytoma also share common clinical features and histopathologic features. Specifically, they both occur in the same age group and at similar sites, the thigh being the most common site for both. The histopathologic subtypes of ESOS may have prognostic significance, but there have been varying conclusions.
CASE REPORT An 83-year-old white woman with no history of trauma or irradiation had a tender swelling in the right breast develop. An ultrasound and mammogram performed at that time demonstrated a solid mass with irregular borders in the right lateral breast. An excisional biopsy was subsequently performed, which revealed an osteoclastic osteosarcoma of the right breast situated within the fibrofatty tissue and the musculature of the breast. The neoplasm was 3.5 cm in its greatest diameter. Immunohistochemical stains with cytokeratin, c-erb B2, estrogen, and progesterone receptors showed no reactivity. Further workup, including chest radiograph, total body bone scan, and computed tomography scan of the abdomen and pelvis, revealed no metastasis. The patient received preoperative doxorubicin. Subsequently, a modified mastectomy of the right breast was performed with sampling of 5 axillary lymph nodes, which did not show evidence of lymphatic
Fig 1. Erythematous tender nodule on posterior aspect of scalp.
metastasis. A follow-up mammogram and chest radiograph performed 1 year later revealed no new lesions. Eighteen months after the initial diagnosis, the patient presented with a gradually enlarging tender erythematous nodule on the posterior aspect of her scalp (Fig 1). An excisional biopsy specimen revealed an ill-defined neoplasm extending from the papillary portion of the dermis to the subcutaneous fat (Fig 2, A). Microscopic findings were very similar to the primary neoplasm. The sarcomatous component consisted of oval to polygonal cells with pleomorphic nuclei and abundant faintly basophilic cytoplasm (Fig 2, C). Numerous atypical and typical mitotic figures were present, varying from 2 to 7 per high-power field (36 per 10 high-power field). The number of osteoclast-like giant cells varied, numerous in some fields to sparse in others. The matrix consisted of irregularly distributed osteoid and fragmented trabeculae of immature bone with varying degrees of mineralization, compatible with osteoblastic subtype (Fig 2, B). Two months after the discovery of the metastatic scalp nodule, the patient died of metastases of the sarcoma, which were present in the lungs, liver, spine, pelvis, and the brain.
DISCUSSION ESOS of the breast is a biologically aggressive neoplasm with frequent recurrences and early hematogenous spread to other organs.16 The metastasis to the scalp in this patient occurred 18 months after the initial diagnosis, presumably hematogenously. Although no evidence of metastasis was present in the lung at the time of diagnosis or 1 year later, it is likely that micrometastases were present in the lung before the evidence of skin metastasis. In those patients with osteosarcoma with eventual lung metastasis, occult micrometastases are believed to
126 Case reports
Fig 2. A, Ill-defined neoplasm spanning entire dermis. B, Trabeculae of immature bone and osteoid with neoplastic sarcomatous cells. C, Osteoclast-like giant cells and sarcomatous cells with multiple mitosis.
be present in 80% to 90% of the cases at the time of diagnosis.17,18 In the largest retrospective study of primary breast ESOS (n ⫽ 50), 41% of the patients had metastasis develop at a mean of 14.5 months from the time of diagnosis, most commonly to the lung.16 In this series, “subcutaneous” metastasis was noted to be present in 4 cases, but the specific details were not mentioned. The size of the primary neoplasm appears to have a prognostic significance,14,16 although not every study supports this observation.13 Survival outcome was significantly better in those patients with primary lesions
J AM ACAD DERMATOL JULY 2003
less than 5 cm. Despite having a small-sized neoplasm, preoperative chemotherapy, and mastectomy with axillary node dissection, this patient had multinodular metastases to the lung, liver, bone, brain, and skin, attesting to the aggressive biologic behavior with early hematogenous metastatic spread of ESOS. There have been inconsistent observations on the prognostic significance of the different histopathologic subtypes. In 1 series, patients with the fibroblastic subtype fared better than the rest,10 whereas in another series, patients with the chondroblastic subtype fared the best,13 and in still another 2 series, there was no difference.12,14 Patients with fibroblastic subtype had the best survival outcome in the largest reported series of mammary ESOS.16 Both the metastatic and the primary lesions in this patient had similar histopathologic features, namely, giant-cellrich with a matrix resembling the osteoblastic subtype, which was found not to have a favorable prognosis in all of the retrospective study series. In both sites, the sarcomatous component consisted of highly pleomorphic cells with numerous atypical and typical mitotic figures, reflective of its aggressive biologic behavior. Of the estimated 1,220,100 new cancers for the year 2000, sarcomas of bones and joints contribute only 2500 (0.2%) cases.19 ESOS is even rarer, composing less than 5% of the osteosarcomas.12,20 Therefore, metastases to the skin, whether from SOS and ESOS, appear to be an exceedingly uncommon phenomenon, as there is a paucity of reported cases in the medical literature. However, in 1 series, 19% of the sarcomas of bone and soft tissue had metastases to the skin, but specific sarcomas were not mentioned.21 Although there has been 1 reported case of metastasis to the oral cavity,22 this is the first reported case of skin metastasis of an ESOS. The scalp nodule in this case was the first sign of widespread metastases reflective of its early metastatic potential and apparent aggressive biologic behavior. Scalp was also the site of metastasis in 2 of 3 case reports of skin metastasis of SOS.5,7 The 2 patients also had concomitant metastasis to the lung. Thus, skin metastasis of an osteosarcoma, whether SOS or ESOS, may be an ominous sign of widespread disease. As the population of older age group increases, ESOS will become more prevalent,23 and more skin metastasis of this rare sarcoma may be observed. REFERENCES 1. Inwards Y, Unni KK. Bone tumors. In: Sternberg SS, Antonioli DA, Carter D, Mills SE, Oberman HA, editors. Diagnostic surgical pathology. Vol 1. 3rd ed. Philadelphia: Lippincott Williams and Wilkins; 1999. p. 263-315. 2. Jaffe N. Chemotherapy for malignant bone tumors. Orthop Clin North Am 1989;20:487-503.
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3. Cancer facts and figures 2000. Atlanta: American Cancer Society Inc; 2000. p. 21. 4. Jeffree GM, Price CHG, Sissons HA. The metastatic patterns of osteosarcoma. Br J Cancer 1975;32:87-107. 5. Finnerud CW. Ossifying sarcoma of the skin metastatic from ossifying sarcoma of the humerus. Arch Dermatol 1924;10:56-62. 6. Myhand RC, Hung PH, Caldwell JB, James WD, Sau P, Hargis JB. Osteogenic sarcoma with skin metastases. J Am Acad Dermatol 1995;32:803-5. 7. Setoyama M, Kanda A, Kanzaki T. Cutaneous metastasis of an osteosarcoma: a case report. Am J Dermatopathol 1996;18:629-32. 8. Kuo TT. Primary osteosarcoma of the skin. J Cutan Pathol 1992; 19:151-5. 9. Logue JP, Cairnduff F. Radiation induced extraskeletal osteosarcoma. Br J Radiol 1991;64:171-2. 10. Chung EB. Enzinger FM. Extraskeletal osteosarcoma. Cancer 1987;60:1132-42. 11. Sordillo PP, Hajdu SI, Magill GB, Golbey RB. Extraosseous osteogenic sarcoma: a review of 48 patients. Cancer 1983;51:72734. 12. Lidang Jensen M, Schumacher B, Myhre Jensen O, Steen Nielsen O, Keller J. Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. Am J Surg Pathol 1998;22:588-94. 13. Lee JY, Fetsch JF, Wasdhal DA, Lee BP, Pritchard DJ, Nascimento AG. A review of 40 patients with extraskeletal osteosarcoma. Cancer 1995;76:2253-9. 14. Bane BL, Evans HL, Ro JY, Carrasco CH, Grignon DJ, Benjamin RS,
15. 16.
17.
18.
19. 20.
21. 22.
23.
et al. Extraskeletal osteosarcoma: a clinicopathologic review of 26 cases. Cancer 1990;65:2762-70. Dorfman HD, Czerniak B. Osteosarcoma. In: Bone tumors. St Louis: Mosby Inc; 1998. p. 128-252. Silver SA, Tavassoli FA. Primary osteogenic sarcoma of the breast: a clinicopathologic analysis of 50 cases. Am J Surg Pathol 1998;22:925-33. Jaffe N. Metastases in malignant childhood tumors–the role of “adjuvant” therapy and the utility of multidisciplinary treatment. Semin Oncol 1977;4:177-26. Pearson M. Historical perspective of the treatment of osteosarcoma: an interview with Dr Norman Jaffe. J Pediatr Oncol Nurs 1998;15:90-4. Cancer facts and figures 2000. Atlanta: American Cancer Society Inc; 2000. p. 4-5. Allan CJ, Soule EH. Osteogenic sarcoma of the somatic soft tissues: clinicopathologic study of 26 cases and review of literature. Cancer 1971;27:1121-33. Spencer PS, Helm TN. Skin metastases in cancer patients. Cutis 1987;39:119-21. Shapiro R, Reichman L, Geltzoff C, Weiss A. Osteosarcoma of breast metastasizing to the oral cavity. Oral Surg Oral Med Oral Pathol 1967;23:58-61. Huvos AG. Osteogenic sarcoma of bones and soft tissues in older persons: a clinicopathologic analysis of 117 patients older than 60 years. Cancer 1986;57:1442-9.
may predispose to trauma, hence, resulting in tissue injury. First, the lesion on the ear is located at a point between a larger and smaller part of helix. This point of the ear probably sustains the largest shearing stress during gestation. Secondly, no subcutaneous tissue in the lateral ear insulates it from trauma. Thirdly, only small blood vessels supply nourishment to epidermis, dermis, and perichondrium. So even minor trauma to the ear may induce ischemic injury. This then may initiate the process of calcification in the tissue. For these reasons we think that calcinosis on the ear in the exact same location is not coincidental and should be a distinct group of calcinosis. We speculate that this form of calcinosis cutis is induced by trauma during gestation. The minor bending of the ear can cause enough trauma to result in calcification. In this way, congenital calcinosis cutis of the ear should be regarded as one type of dystrophic calcification.
J AM ACAD DERMATOL JULY 2003
REFERENCES 1. Winer LH. Solitary nodular calcification of the skin. Arch Dermatol Syph 1952;66:204-11. 2. Mehregan AH. Calcinosis cutis: a review of the clinical forms and report of 75 cases. Semin Dermatol 1984;3:53-61. 3. Younger B, Swan JW. Solitary congenital calcified nodule of the ear. Am J Dermatopathol 1982;4:377-80. 4. Mallory SB, Tryka AF. Solitary congenital nodule of the ear. Arch Dermatol 1988;124:769-74. 5. Lucky AW, Prendiville JS. Solitary congenital nodule on the ear of an infant. Pediatr Dermatol 1993;10:88-90. 6. Evans MJ, Blessing K, Gray ES. Subepidermal calcified nodule in children: a clinicopathologic study of 21 cases. Pediatr Dermatol 1995;12:307-10. 7. Sell EJ, Hansen RC, Struck-Pierce S. Calcified nodules on the heel: a complication of neonatal intensive care. J Pediatr 1980;96: 473-5. 8. Speer ME, Rudolph AJ. Calcification of superficial scalp veins secondary to intravenous infusion of sodium bicarbonate and calcium chloride. Cutis 1983;32:65-6. 9. Wiley HE, Eaglstein WE. Calcinosis cutis in children following electroencephalography. JAMA 1979;242:455-6. 10. Johnson RC, Fitzpatrick JE, Hahn DE. Calcinosis cutis following electromyographic examination. Cutis 1993;52:161-4.
A case of extraskeletal osteosarcoma with metastasis to the skin Seana P. Covello, MD, Tatyana R. Humphreys, MD, and Jason B. Lee, MD Philadelphia, Pennsylvania We present an 83-year-old woman with extraskeletal osteosarcoma (ESOS) of the breast who developed metastasis to the scalp. Skeletal osteosarcoma is the most common primary malignant neoplasm of the bone, predominantly occurring in the metaphysis of the long bones of adolescents and young adults. ESOS, in contrast, occurs primarily in the fifth and sixth decades of life, most commonly in the soft tissue of the thigh. Although the lung is overwhelmingly the most common site of metastasis for both skeletal osteosarcoma and ESOS, the skin is an uncommon metastatic site with only a few reported cases. Metastasis of ESOS to the skin is an exceedingly infrequent phenomenon, which may be a sign of widespread metastases foreboding a grim prognosis. (J Am Acad Dermatol 2003;49:124-7.)
O
steosarcoma is a malignant neoplasm of the bone in which the mesenchymal neoplastic cells have the ability to produce osteoid or immature bone. It is the most common primary malignant neoplasm of the bone, predominantly oc-
From the Department of Dermatology and Cutaneous Biology, Jefferson Medical College. Funding sources: None. Conflict of interest: None identified. Reprint requests: Jason B. Lee, MD, Jefferson Medical College, Department of Dermatology and Cutaneous Biology, 833 Chestnut St, Suite 740, Philadelphia, PA 19107. E-mail: jason.b.lee@ mail.tju.edu. Copyright © 2003 by the American Academy of Dermatology, Inc. 0190-9622/2003/$30.00 ⫹ 0 doi:10.1067/mjd.2003.297
curring in the metaphysis of the long bones of adolescents and young adults, corresponding to the peak period of skeletal growth.1 Before the advent of chemotherapy, specifically doxorubicin, highdose methotrexate, cisplatin, and ifosfamide,2 the 5-year survival was dismal at 20%, but now has risen to 63%.3 The lung is the most common site of metastasis4 with very few reported cases of metastasis to the skin.5-7 Rarely, osteosarcomas occur at sites other than the bone, the so-called extraskeletal osteosarcoma (ESOS). ESOS has been reported to occur in virtually every organ, including the skin.8,9 However, the most common site is the thigh, which usually presents as a deep-seated, soft-tissue mass.10 In contrast to skeletal osteosarcoma (SOS), ESOS is
Case reports 125
J AM ACAD DERMATOL VOLUME 49, NUMBER 1
primarily observed in the fifth and sixth decades of life.11,12 ESOS may develop at sites of trauma (6%24%) and areas of radiation (4%-10%).10-14 ESOS has a 5-year survival that ranges from 25% to 37%.12-14 The lower survival outcome compared with SOS may be partly explained by the older age group. Radical wide excision affords the best chance for cure because of its high recurrence rate and early metastasis. Similar to SOS, the lung is overwhelmingly the most common site of metastasis, whereas metastasis to the skin appears to be exceedingly rare. There are 2 microscopic components of SOS and ESOS, namely sarcomatous neoplastic cells and extracellular matrix, which consist of osteoid and/or immature bone. Three main histopathologic subtypes are recognized depending on this matrix product: osteoblastic; chondroblastic; and fibroblastic.15 When osteoclast-like cells are abundant, these neoplasms are descriptively designated as giant-cell-rich or osteoclastic osteosarcomas. When bone formation is inconspicuous in the fibroblastic subtype, the histopathologic findings may be difficult to distinguish from malignant fibrous histiocytoma,10 but alkaline phosphatase staining on fresh tissue may be a helpful distinguishing marker, which stains ESOS prominently.12 Fibroblastic ESOS and malignant fibrous histiocytoma also share common clinical features and histopathologic features. Specifically, they both occur in the same age group and at similar sites, the thigh being the most common site for both. The histopathologic subtypes of ESOS may have prognostic significance, but there have been varying conclusions.
CASE REPORT An 83-year-old white woman with no history of trauma or irradiation had a tender swelling in the right breast develop. An ultrasound and mammogram performed at that time demonstrated a solid mass with irregular borders in the right lateral breast. An excisional biopsy was subsequently performed, which revealed an osteoclastic osteosarcoma of the right breast situated within the fibrofatty tissue and the musculature of the breast. The neoplasm was 3.5 cm in its greatest diameter. Immunohistochemical stains with cytokeratin, c-erb B2, estrogen, and progesterone receptors showed no reactivity. Further workup, including chest radiograph, total body bone scan, and computed tomography scan of the abdomen and pelvis, revealed no metastasis. The patient received preoperative doxorubicin. Subsequently, a modified mastectomy of the right breast was performed with sampling of 5 axillary lymph nodes, which did not show evidence of lymphatic
Fig 1. Erythematous tender nodule on posterior aspect of scalp.
metastasis. A follow-up mammogram and chest radiograph performed 1 year later revealed no new lesions. Eighteen months after the initial diagnosis, the patient presented with a gradually enlarging tender erythematous nodule on the posterior aspect of her scalp (Fig 1). An excisional biopsy specimen revealed an ill-defined neoplasm extending from the papillary portion of the dermis to the subcutaneous fat (Fig 2, A). Microscopic findings were very similar to the primary neoplasm. The sarcomatous component consisted of oval to polygonal cells with pleomorphic nuclei and abundant faintly basophilic cytoplasm (Fig 2, C). Numerous atypical and typical mitotic figures were present, varying from 2 to 7 per high-power field (36 per 10 high-power field). The number of osteoclast-like giant cells varied, numerous in some fields to sparse in others. The matrix consisted of irregularly distributed osteoid and fragmented trabeculae of immature bone with varying degrees of mineralization, compatible with osteoblastic subtype (Fig 2, B). Two months after the discovery of the metastatic scalp nodule, the patient died of metastases of the sarcoma, which were present in the lungs, liver, spine, pelvis, and the brain.
DISCUSSION ESOS of the breast is a biologically aggressive neoplasm with frequent recurrences and early hematogenous spread to other organs.16 The metastasis to the scalp in this patient occurred 18 months after the initial diagnosis, presumably hematogenously. Although no evidence of metastasis was present in the lung at the time of diagnosis or 1 year later, it is likely that micrometastases were present in the lung before the evidence of skin metastasis. In those patients with osteosarcoma with eventual lung metastasis, occult micrometastases are believed to
126 Case reports
Fig 2. A, Ill-defined neoplasm spanning entire dermis. B, Trabeculae of immature bone and osteoid with neoplastic sarcomatous cells. C, Osteoclast-like giant cells and sarcomatous cells with multiple mitosis.
be present in 80% to 90% of the cases at the time of diagnosis.17,18 In the largest retrospective study of primary breast ESOS (n ⫽ 50), 41% of the patients had metastasis develop at a mean of 14.5 months from the time of diagnosis, most commonly to the lung.16 In this series, “subcutaneous” metastasis was noted to be present in 4 cases, but the specific details were not mentioned. The size of the primary neoplasm appears to have a prognostic significance,14,16 although not every study supports this observation.13 Survival outcome was significantly better in those patients with primary lesions
J AM ACAD DERMATOL JULY 2003
less than 5 cm. Despite having a small-sized neoplasm, preoperative chemotherapy, and mastectomy with axillary node dissection, this patient had multinodular metastases to the lung, liver, bone, brain, and skin, attesting to the aggressive biologic behavior with early hematogenous metastatic spread of ESOS. There have been inconsistent observations on the prognostic significance of the different histopathologic subtypes. In 1 series, patients with the fibroblastic subtype fared better than the rest,10 whereas in another series, patients with the chondroblastic subtype fared the best,13 and in still another 2 series, there was no difference.12,14 Patients with fibroblastic subtype had the best survival outcome in the largest reported series of mammary ESOS.16 Both the metastatic and the primary lesions in this patient had similar histopathologic features, namely, giant-cellrich with a matrix resembling the osteoblastic subtype, which was found not to have a favorable prognosis in all of the retrospective study series. In both sites, the sarcomatous component consisted of highly pleomorphic cells with numerous atypical and typical mitotic figures, reflective of its aggressive biologic behavior. Of the estimated 1,220,100 new cancers for the year 2000, sarcomas of bones and joints contribute only 2500 (0.2%) cases.19 ESOS is even rarer, composing less than 5% of the osteosarcomas.12,20 Therefore, metastases to the skin, whether from SOS and ESOS, appear to be an exceedingly uncommon phenomenon, as there is a paucity of reported cases in the medical literature. However, in 1 series, 19% of the sarcomas of bone and soft tissue had metastases to the skin, but specific sarcomas were not mentioned.21 Although there has been 1 reported case of metastasis to the oral cavity,22 this is the first reported case of skin metastasis of an ESOS. The scalp nodule in this case was the first sign of widespread metastases reflective of its early metastatic potential and apparent aggressive biologic behavior. Scalp was also the site of metastasis in 2 of 3 case reports of skin metastasis of SOS.5,7 The 2 patients also had concomitant metastasis to the lung. Thus, skin metastasis of an osteosarcoma, whether SOS or ESOS, may be an ominous sign of widespread disease. As the population of older age group increases, ESOS will become more prevalent,23 and more skin metastasis of this rare sarcoma may be observed. REFERENCES 1. Inwards Y, Unni KK. Bone tumors. In: Sternberg SS, Antonioli DA, Carter D, Mills SE, Oberman HA, editors. Diagnostic surgical pathology. Vol 1. 3rd ed. Philadelphia: Lippincott Williams and Wilkins; 1999. p. 263-315. 2. Jaffe N. Chemotherapy for malignant bone tumors. Orthop Clin North Am 1989;20:487-503.
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J AM ACAD DERMATOL VOLUME 49, NUMBER 1
3. Cancer facts and figures 2000. Atlanta: American Cancer Society Inc; 2000. p. 21. 4. Jeffree GM, Price CHG, Sissons HA. The metastatic patterns of osteosarcoma. Br J Cancer 1975;32:87-107. 5. Finnerud CW. Ossifying sarcoma of the skin metastatic from ossifying sarcoma of the humerus. Arch Dermatol 1924;10:56-62. 6. Myhand RC, Hung PH, Caldwell JB, James WD, Sau P, Hargis JB. Osteogenic sarcoma with skin metastases. J Am Acad Dermatol 1995;32:803-5. 7. Setoyama M, Kanda A, Kanzaki T. Cutaneous metastasis of an osteosarcoma: a case report. Am J Dermatopathol 1996;18:629-32. 8. Kuo TT. Primary osteosarcoma of the skin. J Cutan Pathol 1992; 19:151-5. 9. Logue JP, Cairnduff F. Radiation induced extraskeletal osteosarcoma. Br J Radiol 1991;64:171-2. 10. Chung EB. Enzinger FM. Extraskeletal osteosarcoma. Cancer 1987;60:1132-42. 11. Sordillo PP, Hajdu SI, Magill GB, Golbey RB. Extraosseous osteogenic sarcoma: a review of 48 patients. Cancer 1983;51:72734. 12. Lidang Jensen M, Schumacher B, Myhre Jensen O, Steen Nielsen O, Keller J. Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. Am J Surg Pathol 1998;22:588-94. 13. Lee JY, Fetsch JF, Wasdhal DA, Lee BP, Pritchard DJ, Nascimento AG. A review of 40 patients with extraskeletal osteosarcoma. Cancer 1995;76:2253-9. 14. Bane BL, Evans HL, Ro JY, Carrasco CH, Grignon DJ, Benjamin RS,
15. 16.
17.
18.
19. 20.
21. 22.
23.
et al. Extraskeletal osteosarcoma: a clinicopathologic review of 26 cases. Cancer 1990;65:2762-70. Dorfman HD, Czerniak B. Osteosarcoma. In: Bone tumors. St Louis: Mosby Inc; 1998. p. 128-252. Silver SA, Tavassoli FA. Primary osteogenic sarcoma of the breast: a clinicopathologic analysis of 50 cases. Am J Surg Pathol 1998;22:925-33. Jaffe N. Metastases in malignant childhood tumors–the role of “adjuvant” therapy and the utility of multidisciplinary treatment. Semin Oncol 1977;4:177-26. Pearson M. Historical perspective of the treatment of osteosarcoma: an interview with Dr Norman Jaffe. J Pediatr Oncol Nurs 1998;15:90-4. Cancer facts and figures 2000. Atlanta: American Cancer Society Inc; 2000. p. 4-5. Allan CJ, Soule EH. Osteogenic sarcoma of the somatic soft tissues: clinicopathologic study of 26 cases and review of literature. Cancer 1971;27:1121-33. Spencer PS, Helm TN. Skin metastases in cancer patients. Cutis 1987;39:119-21. Shapiro R, Reichman L, Geltzoff C, Weiss A. Osteosarcoma of breast metastasizing to the oral cavity. Oral Surg Oral Med Oral Pathol 1967;23:58-61. Huvos AG. Osteogenic sarcoma of bones and soft tissues in older persons: a clinicopathologic analysis of 117 patients older than 60 years. Cancer 1986;57:1442-9.