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Metastatic thymic carcinoma in a digit: A case report
Metastatic Thymic Carcinoma in a Digit: A Case Report Fintan J. Shannon, MD, Cristina R. Antonescu, MD, Edward A. Athanasian, MD, New York, NY Acrometastases are a rare but important clinical entity. We present the case of a 54-year-old man with a metastasis to a digit from a primary thymic carcinoma. The prognostic implications of such a diagnosis are discussed. (J Hand Surg 2000;25A:1169 –1172. Copyright © 2000 by the American Society for Surgery of the Hand.) Key words: Acrometastasis, phalanx, thymic carcinoma.
Thymic carcinomas are malignant neoplasms of the thymus characterized by extensive infiltration into surrounding tissues and obvious cytologic atypia, associated with frequent areas of necrosis. Many different histologic types of thymic carcinoma have been characterized. Most of them occur in adult men; they rarely occur in children. The microscopic finding of keratinization correlates with a good prognosis. The other histologic types, including undifferentiated (anaplastic) carcinoma, are usually associated with a fatal outcome.1–3 Symptoms include weight loss, cough, and shoulder discomfort.4 Myasthenia gravis and other paraneoplastic syndromes commonly reported in patients with thymomas are not usually associated with thymic carcinoma. The specific etiology of thymic carcinoma is unknown; however, increasing evidence suggests that EpsteinBarr virus plays a role in the development of a lymphoepithelioma-like carcinoma of the thymus gland.5 The treatment of these tumors warrants a From the Departments of Surgery/Orthopaedics and Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY. Received for publication April 25, 2000; accepted in revised form May 7, 2000. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Edward A. Athanasian, MD, Department of Surgery/Orthopaedics, Suite A675, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10021. Copyright © 2000 by the American Society for Surgery of the Hand 0363-5023/00/25A06-0003$3.00/0 doi: 10.1053/jhsu.2000.17863
multidisciplinary approach that includes surgery, radiation therapy, and chemotherapy.6 The combination of cisplatin, vinblastine, and bleomycin is considered effective for these neoplasms. Metastases to the pleura, lungs, and bone are common. We present the case of a thymic carcinoma with metastasis to a digit.
Case Report A 54-year-old man was referred to the orthopedic service following an 8-week history of a painful swelling on the volar aspect of the right ring finger. He was unable to flex the distal interphalangeal joint but was otherwise well. Examination revealed a firm, fixed, nontender, purple swelling on the volar aspect of the distal phalanx. The dimensions were 1.0 ⫻ 2.0 ⫻ 0.5 cm. Neurovascular examination of the digit was normal. A short longitudinal scar was present over the swelling. Incision and drainage was performed for presumed infection 1 week before our evaluation. Seven years before presentation the patient underwent excision of a poorly differentiated thymic carcinoma. A solitary cerebellar metastasis was resected and bilateral pleural and pulmonary metastases were present at the time of our evaluation. The evaluating radiologist felt that the plain x-rays and technetium bone scan were consistent with osteomyelitis of the distal phalanx (Fig. 1). The patient was admitted for biopsy and treatment. A biopsy was performed under regional anesthesia using a 5-mm longitudinal incision on the radial The Journal of Hand Surgery 1169
1170
Shannon, Antonescu, and Athanasian / Metastatic Thymic Carcinoma in a Digit
Figure 1. Anteroposterior (A) and lateral (B) radiographs of the right index finger demonstrating soft tissue swelling and bony erosion of the distal phalanx.
aspect of the digit. Frozen section analysis revealed metastatic carcinoma. Amputation through the proximal interphalangeal joint was performed. Soft tissue coverage was achieved with the creation of a short palmar skin flap and a larger dorsal skin flap. The patient’s postoperative recovery was uneventful. Final pathologic analysis confirmed metastatic poorly differentiated carcinoma involving the dermis, subcutaneous tissue, and distal phalanx that was morphologically similar to the primary thymic tumor (Figs. 2, 3). Surgical margins were negative. The patient died 2 months after amputation due to progression of the widespread metastases.
tissue or skeletal diseases in the hand. Our patient was treated for suspected osteomyelitis and felon with antibiotics and attempted incision and drainage
Discussion Metastases to the bones and soft tissues of the hand are rare, representing 0.1% of all metastatic lesions.7–12 The most common sites of origin are the lung, kidney, and breast, with the lung representing more than 40% of the reported primary tumors that metastasize to the hand.10, 13–17 Acrometastases may be the first manifestation of an occult malignancy13,18 and can mimic other more common soft
Figure 2. Microscopic appearance of the poorly differentiated thymic carcinoma showing an alveolar pattern of growth of the small undifferentiated tumor cells in the anterior mediastinum. (Hematoxylin-eosin stain; medium power.)
The Journal of Hand Surgery / Vol. 25A No. 6 November 2000 1171
Figure 3. Metastatic undifferentiated thymic carcinoma (bottom right) in the phalanx showing bone destruction (bottom left) in the vicinity of the nail bed (top). (Hematoxylin-eosin stain; low power.)
before an orthopedic evaluation. Patients with acrometastases are typically in their fifth decade of life or older and present with a short history of pain, swelling, and erythema that may mimic infection. Consideration and awareness of the potential for acrometastases in patients with a known history of cancer or in older patients is essential to avoid misdiagnosis and delay of treatment. The diagnosis of metastatic disease in the hand generally carries a poor prognosis, with most patients surviving less than 6 months.17,19 –23 In 1958, Kerin24 reviewed metastatic tumors of the hand and stated that, “The lethal implication that a metastatic malignant tumour to the hand carries is indicated by the fact that nowhere in the literature was the problem of therapy discussed. In all of the cases it was a foregone conclusion that death would supervene and some of the reports stated that some of the individuals were either dead or dying at the time of writing.” Advances in oncology may prolong survival; however, metastases to the hand usually suggests disseminated widespread disease. Healey et al13 and others22,25 noted that pulmonary involvement was a common denominator in many of their patients and speculated that in patients with pulmonary metastases the source of the acrometastases may be the secondary tumor (pulmonary) rather than the primary tumor. In general the goal of treatment should be palliation, but wide excision or amputation of apparently isolated metastatic hand lesions can produce long disease-free intervals. Recent analysis of patients
with solitary skeletal renal cell carcinoma metastases demonstrated the potential for long term-survival following wide excision.26 Patients presenting with solitary renal cell carcinoma acrometastases should be treated with wide excision or amputation as this has the potential to be life saving. Relief of pain in symptomatic distal hand lesions with preservation of maximal hand function is best achieved with amputation through the more proximal joint. When the lesion is more proximal, curettage, cementation, and radiotherapy may be effective. The justification for adjuvant therapy is based on the individual patient’s overall disease status and the specific disease.
References 1. Walker AN, Mills SE, Fechner RE. Thymomas and thymic carcinomas. Semin Diagn Pathol 1990;7:250 –265. 2. Wick MR, Weiland LH, Scheithauer BW, Bernatz PE. Primary thymic carcinomas. Am J Surg Pathol 1982;6: 613– 630. 3. Snover DC, Levine GD, Rosai J. Thymic carcinoma: five distinctive histological variants. Am J Surg Pathol 1982; 6:451– 470. 4. Aisner J, Antman KH, Belani CP. Pleura and mediastinum. In: Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE, eds. Clinical oncology. 2nd ed. New York: Churchill Livingstone, 2000:1497–1498. 5. Leyvraz S, Henle W, Chahinian AP, et al. Association of Epstein-Barr virus with thymic carcinoma. N Engl J Med 1985;312:1296 –1299. 6. Thomas CR Jr, Bonomi P. Mediastinal tumors. Curr Opin Oncol1990;2:359 –367. 7. Bricout PB. Acrometastases. J Natl Med Assoc1981;73: 325–329. 8. Clain A. Secondary malignant disease of bone. Br J Cancer 1965;19:15–29. 9. Gold GL, Reefe WE. Carcinoma and metastases to the bones of the hand. JAMA 1963;184:237–239. 10. Mangini U. Tumors of the skeleton of the hand. Bull Hosp Jt Dis Orthop Inst 1967;28:61–103. 11. Martin KA, Dove AF. Metastatic carcinoma of the hand. Hand 1983;15:343–346. 12. Kumar PP. Metastases to the bones of the hand. J Natl Med Assoc 1975;67:275–276. 13. Healey JH, Turnbull ADM, Miedema B, Lane JM. Acrometastases: a study of twenty-nine patients with osseous involvement of the hands and feet. J Bone Joint Surg 1986;68A:743–746. 14. Amadio PC, Lombardi RM. Metastatic tumors of the hand. J Hand Surg 1987;12:311–316. 15. Kerin R. Metastatic tumors of the hand: a review of the literature. J Bone Joint Surg 1983;65A:1331–1335. 16. Fragiadakis EG, Panayotopoulos G. Metastatic carcinoma of the hand. Hand 1972;4:268 –272. 17. Morris DM, House HC. The significance of metastasis to
1172
18. 19. 20. 21.
22.
Shannon, Antonescu, and Athanasian / Metastatic Thymic Carcinoma in a Digit
the bones and soft tissues of the hand. J Surg Oncol 1985;28:146 –150. Abrahams TG. Occult malignancy presenting as metastatic disease to the hand and wrist. Skeletal Radiol 1995;24:135–137. Wu KK, Guise ER. Metastatic tumors of the hand: a report of six cases. J Hand Surg 1978;3:271–276. Basora J, Fery A. Metastatic malignancy of the hand. Clin Orthop 1975;108:182–186 Bryan RS, Soule EH, Dobyns JH, Pritchard DJ, Linscheid RL. Metastatic lesions of the hand and forearm. Clin Orthop 1974;101:167–170. Greene MH. Metastasis of pulmonary carcinoma to the
23. 24. 25.
26.
phalanges of the hand. J Bone Joint Surg 1957;39:972– 975. Sim FH, Pritchard DJ. Metastatic disease in upper extremity. Clin Orthop 1982;169:83–94. Kerin R. Metastatic tumors of the hand. J Bone Joint Surg 1958;40A:263–277. Nagendran T, Patel MN, Gaillard WE, Imm F, Walker M. Metastatic bronchogenic carcinoma to the bones of the hand. Cancer 1980;45:824 – 828. Althausen P, Althausen A, Jennings LC, Mankin HJ. Prognostic factors and surgical treatment of osseous metastases secondary to renal cell carcinoma. Cancer 1997;80:1103–1109.
Thymic carcinomas are malignant neoplasms of the thymus characterized by extensive infiltration into surrounding tissues and obvious cytologic atypia, associated with frequent areas of necrosis. Many different histologic types of thymic carcinoma have been characterized. Most of them occur in adult men; they rarely occur in children. The microscopic finding of keratinization correlates with a good prognosis. The other histologic types, including undifferentiated (anaplastic) carcinoma, are usually associated with a fatal outcome.1–3 Symptoms include weight loss, cough, and shoulder discomfort.4 Myasthenia gravis and other paraneoplastic syndromes commonly reported in patients with thymomas are not usually associated with thymic carcinoma. The specific etiology of thymic carcinoma is unknown; however, increasing evidence suggests that EpsteinBarr virus plays a role in the development of a lymphoepithelioma-like carcinoma of the thymus gland.5 The treatment of these tumors warrants a From the Departments of Surgery/Orthopaedics and Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY. Received for publication April 25, 2000; accepted in revised form May 7, 2000. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Edward A. Athanasian, MD, Department of Surgery/Orthopaedics, Suite A675, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10021. Copyright © 2000 by the American Society for Surgery of the Hand 0363-5023/00/25A06-0003$3.00/0 doi: 10.1053/jhsu.2000.17863
multidisciplinary approach that includes surgery, radiation therapy, and chemotherapy.6 The combination of cisplatin, vinblastine, and bleomycin is considered effective for these neoplasms. Metastases to the pleura, lungs, and bone are common. We present the case of a thymic carcinoma with metastasis to a digit.
Case Report A 54-year-old man was referred to the orthopedic service following an 8-week history of a painful swelling on the volar aspect of the right ring finger. He was unable to flex the distal interphalangeal joint but was otherwise well. Examination revealed a firm, fixed, nontender, purple swelling on the volar aspect of the distal phalanx. The dimensions were 1.0 ⫻ 2.0 ⫻ 0.5 cm. Neurovascular examination of the digit was normal. A short longitudinal scar was present over the swelling. Incision and drainage was performed for presumed infection 1 week before our evaluation. Seven years before presentation the patient underwent excision of a poorly differentiated thymic carcinoma. A solitary cerebellar metastasis was resected and bilateral pleural and pulmonary metastases were present at the time of our evaluation. The evaluating radiologist felt that the plain x-rays and technetium bone scan were consistent with osteomyelitis of the distal phalanx (Fig. 1). The patient was admitted for biopsy and treatment. A biopsy was performed under regional anesthesia using a 5-mm longitudinal incision on the radial The Journal of Hand Surgery 1169
1170
Shannon, Antonescu, and Athanasian / Metastatic Thymic Carcinoma in a Digit
Figure 1. Anteroposterior (A) and lateral (B) radiographs of the right index finger demonstrating soft tissue swelling and bony erosion of the distal phalanx.
aspect of the digit. Frozen section analysis revealed metastatic carcinoma. Amputation through the proximal interphalangeal joint was performed. Soft tissue coverage was achieved with the creation of a short palmar skin flap and a larger dorsal skin flap. The patient’s postoperative recovery was uneventful. Final pathologic analysis confirmed metastatic poorly differentiated carcinoma involving the dermis, subcutaneous tissue, and distal phalanx that was morphologically similar to the primary thymic tumor (Figs. 2, 3). Surgical margins were negative. The patient died 2 months after amputation due to progression of the widespread metastases.
tissue or skeletal diseases in the hand. Our patient was treated for suspected osteomyelitis and felon with antibiotics and attempted incision and drainage
Discussion Metastases to the bones and soft tissues of the hand are rare, representing 0.1% of all metastatic lesions.7–12 The most common sites of origin are the lung, kidney, and breast, with the lung representing more than 40% of the reported primary tumors that metastasize to the hand.10, 13–17 Acrometastases may be the first manifestation of an occult malignancy13,18 and can mimic other more common soft
Figure 2. Microscopic appearance of the poorly differentiated thymic carcinoma showing an alveolar pattern of growth of the small undifferentiated tumor cells in the anterior mediastinum. (Hematoxylin-eosin stain; medium power.)
The Journal of Hand Surgery / Vol. 25A No. 6 November 2000 1171
Figure 3. Metastatic undifferentiated thymic carcinoma (bottom right) in the phalanx showing bone destruction (bottom left) in the vicinity of the nail bed (top). (Hematoxylin-eosin stain; low power.)
before an orthopedic evaluation. Patients with acrometastases are typically in their fifth decade of life or older and present with a short history of pain, swelling, and erythema that may mimic infection. Consideration and awareness of the potential for acrometastases in patients with a known history of cancer or in older patients is essential to avoid misdiagnosis and delay of treatment. The diagnosis of metastatic disease in the hand generally carries a poor prognosis, with most patients surviving less than 6 months.17,19 –23 In 1958, Kerin24 reviewed metastatic tumors of the hand and stated that, “The lethal implication that a metastatic malignant tumour to the hand carries is indicated by the fact that nowhere in the literature was the problem of therapy discussed. In all of the cases it was a foregone conclusion that death would supervene and some of the reports stated that some of the individuals were either dead or dying at the time of writing.” Advances in oncology may prolong survival; however, metastases to the hand usually suggests disseminated widespread disease. Healey et al13 and others22,25 noted that pulmonary involvement was a common denominator in many of their patients and speculated that in patients with pulmonary metastases the source of the acrometastases may be the secondary tumor (pulmonary) rather than the primary tumor. In general the goal of treatment should be palliation, but wide excision or amputation of apparently isolated metastatic hand lesions can produce long disease-free intervals. Recent analysis of patients
with solitary skeletal renal cell carcinoma metastases demonstrated the potential for long term-survival following wide excision.26 Patients presenting with solitary renal cell carcinoma acrometastases should be treated with wide excision or amputation as this has the potential to be life saving. Relief of pain in symptomatic distal hand lesions with preservation of maximal hand function is best achieved with amputation through the more proximal joint. When the lesion is more proximal, curettage, cementation, and radiotherapy may be effective. The justification for adjuvant therapy is based on the individual patient’s overall disease status and the specific disease.
References 1. Walker AN, Mills SE, Fechner RE. Thymomas and thymic carcinomas. Semin Diagn Pathol 1990;7:250 –265. 2. Wick MR, Weiland LH, Scheithauer BW, Bernatz PE. Primary thymic carcinomas. Am J Surg Pathol 1982;6: 613– 630. 3. Snover DC, Levine GD, Rosai J. Thymic carcinoma: five distinctive histological variants. Am J Surg Pathol 1982; 6:451– 470. 4. Aisner J, Antman KH, Belani CP. Pleura and mediastinum. In: Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE, eds. Clinical oncology. 2nd ed. New York: Churchill Livingstone, 2000:1497–1498. 5. Leyvraz S, Henle W, Chahinian AP, et al. Association of Epstein-Barr virus with thymic carcinoma. N Engl J Med 1985;312:1296 –1299. 6. Thomas CR Jr, Bonomi P. Mediastinal tumors. Curr Opin Oncol1990;2:359 –367. 7. Bricout PB. Acrometastases. J Natl Med Assoc1981;73: 325–329. 8. Clain A. Secondary malignant disease of bone. Br J Cancer 1965;19:15–29. 9. Gold GL, Reefe WE. Carcinoma and metastases to the bones of the hand. JAMA 1963;184:237–239. 10. Mangini U. Tumors of the skeleton of the hand. Bull Hosp Jt Dis Orthop Inst 1967;28:61–103. 11. Martin KA, Dove AF. Metastatic carcinoma of the hand. Hand 1983;15:343–346. 12. Kumar PP. Metastases to the bones of the hand. J Natl Med Assoc 1975;67:275–276. 13. Healey JH, Turnbull ADM, Miedema B, Lane JM. Acrometastases: a study of twenty-nine patients with osseous involvement of the hands and feet. J Bone Joint Surg 1986;68A:743–746. 14. Amadio PC, Lombardi RM. Metastatic tumors of the hand. J Hand Surg 1987;12:311–316. 15. Kerin R. Metastatic tumors of the hand: a review of the literature. J Bone Joint Surg 1983;65A:1331–1335. 16. Fragiadakis EG, Panayotopoulos G. Metastatic carcinoma of the hand. Hand 1972;4:268 –272. 17. Morris DM, House HC. The significance of metastasis to
1172
18. 19. 20. 21.
22.
Shannon, Antonescu, and Athanasian / Metastatic Thymic Carcinoma in a Digit
the bones and soft tissues of the hand. J Surg Oncol 1985;28:146 –150. Abrahams TG. Occult malignancy presenting as metastatic disease to the hand and wrist. Skeletal Radiol 1995;24:135–137. Wu KK, Guise ER. Metastatic tumors of the hand: a report of six cases. J Hand Surg 1978;3:271–276. Basora J, Fery A. Metastatic malignancy of the hand. Clin Orthop 1975;108:182–186 Bryan RS, Soule EH, Dobyns JH, Pritchard DJ, Linscheid RL. Metastatic lesions of the hand and forearm. Clin Orthop 1974;101:167–170. Greene MH. Metastasis of pulmonary carcinoma to the
23. 24. 25.
26.
phalanges of the hand. J Bone Joint Surg 1957;39:972– 975. Sim FH, Pritchard DJ. Metastatic disease in upper extremity. Clin Orthop 1982;169:83–94. Kerin R. Metastatic tumors of the hand. J Bone Joint Surg 1958;40A:263–277. Nagendran T, Patel MN, Gaillard WE, Imm F, Walker M. Metastatic bronchogenic carcinoma to the bones of the hand. Cancer 1980;45:824 – 828. Althausen P, Althausen A, Jennings LC, Mankin HJ. Prognostic factors and surgical treatment of osseous metastases secondary to renal cell carcinoma. Cancer 1997;80:1103–1109.