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A Variant of AESOP Syndrome (Adenopathy and Extensive Skin Patch Overlying a Plasmacytoma) in a Malignant Blue-Cell Tumor

A Variant of AESOP Syndrome (Adenopathy and Extensive Skin Patch Overlying a Plasmacytoma) in a... Report of a Case A 53-year-old man was referred in 1998 for a pruritic eruption on his central chest of 8 months' duration. He reported a sensation of bulging over his lower sternum. On examination, he had a sharply demarcated, indurated, erythematous plaque on the central chest (Figure 1) and bilateral axillary lymphadenopathy. A biopsy specimen showed a nonspecific dermal proliferation of vascular structures (CD34+) with pleomorphic and multinucleated histiocytes (CD68+) and myofibroblasts (Figure 2). Results of immunostaining for S100, factor XIIIA, keratin, estrogen receptor, and progesterone receptor were negative. Tissue cultures were negative. View LargeDownload Figure 1. Sharply demarcated, indurated, erythematous plaque on the bilateral breasts and chest. View LargeDownload Figure 2. Dermal proliferation of vascular structures (CD34+) with pleomorphic and multinucleated histiocytes (CD68+) and myofibroblasts. A magnetic resonance image of the chest demonstrated a 5.6 × 2.0 × 3.0-cm soft-tissue mass involving the lower portion of his sternum and subcutaneous fat. Results of computed tomography–guided fine-needle aspiration showed sheets of round to oval hyperchromatic cells with scant amounts of cytoplasm. Tumor cells were strongly immunoreactive with vimentin but tested negative with CD99, CD79A, λ- and κ-light chains, actin, CD3, CD20, PTAH, cytokeratins AE1/AE3, CAM 5.2, S100, chromogranin, and synaptophysin. The pathologists concluded that the tumor represented a Ewing sarcoma–like, small, blue-cell malignant neoplasm. Findings of serum and urine protein electrophoresis and serum immunoglobulin assays were normal. A bone-marrow aspirate showed no malignancy. Further imaging demonstrated bilateral axillary lymphadenopathy and a single dense sclerotic lesion within his third lumbar vertebra. Axillary lymph node biopsy specimens revealed follicular lymphoid hyperplasia with no evidence of metastatic disease. The lumbar vertebral lesion was considered to be metastatic tumor. He received 4 cycles of chemotherapy consisting of cyclophosphamide, doxorubicin, and vincristine. This was followed by radiotherapy to the sternal tumor and third lumbar vertebral lesion. He received an additional 6 cycles of chemotherapy that consisted of ifosfamide and VP-16 for treatment of residual tumor. His skin lesion on the chest regressed 1 year after chemoradiation therapy, and the axillary lymphadenopathy resolved. Positron-emission tomography 2 years later showed no evidence of tumor. He had no evidence of disease recurrence at 6-year follow-up. Comment The term AESOP syndrome was coined by Lipsker et al.1 The clinical syndrome consists of an extensive violaceous skin patch overlying a solitary plasmacytoma of bone that is associated with regional lymphadenopathy. In the cases reported by Lipsker et al,1 histologic findings of the skin and lymph nodes were nonspecific. Treatment of the plasmacytoma resulted in regression of the skin lesion and enlarged lymph nodes. Herein, we present a similar case of a sharply demarcated, erythematous plaque over the sternum with axillary adenopathy developing over a small blue-cell sarcoma with Ewing-like features, instead of a plasmacytoma. The histologic features of the cutaneous plaque were comparable to the cases described by Lipsker et al,1 which had the hallmark of high density of small dilated vessels throughout the dermis. Regression of the primary tumor resulted in resolution of the cutaneous lesions. To our knowledge, there are only 9 patients in whom plasmacytomas presented with the AESOP syndrome.1-5 There have been no reports of other malignant tumors presenting in a similar fashion. We postulate that such a clinical syndrome is not limited to plasmacytomas but instead represents a paraneoplastic syndrome that can occur with other underlying malignant tumors. Back to top Article Information Correspondence: Dr Foo, Department of Dermatology, University of Utah, 4A330 School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132 (chong.foo@hsc.utah.edu). Conflict of Interest Disclosures: None reported. References 1. Lipsker D, Rondeau M, Massard G, Grosshans E. The AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndrome: report of 4 cases of a new syndrome revealing POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome at a curable stage. Medicine (Baltimore). 2003;82(1):51-5912544710PubMedGoogle ScholarCrossref 2. Gupta RM, Roy DC, Gupta IM, Khanna S. Extramedullary plasmacytoma IgG type I presenting as mediastinal syndrome. Br J Dis Chest. 1974;68(1):65-704819775PubMedGoogle ScholarCrossref 3. Read D, Warlow C. Peripheral neuropathy and solitary plasmacytoma. J Neurol Neurosurg Psychiatry. 1978;41(2):177-184204745PubMedGoogle ScholarCrossref 4. Feddersen RM, Burgdorf W, Foucar K, Elias L, Smith SM. Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation. J Am Acad Dermatol. 1989;21(5, pt 2):1061-10682681292PubMedGoogle ScholarCrossref 5. Weichenthal M, Stemm AV, Ramsauer J, Mensing H, Feller AC, Meigel W. POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. J Am Acad Dermatol. 1999;40(5, pt 2):808-81210321621PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

A Variant of AESOP Syndrome (Adenopathy and Extensive Skin Patch Overlying a Plasmacytoma) in a Malignant Blue-Cell Tumor

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References (6)

Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archdermatol.2012.2858
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Abstract

Report of a Case A 53-year-old man was referred in 1998 for a pruritic eruption on his central chest of 8 months' duration. He reported a sensation of bulging over his lower sternum. On examination, he had a sharply demarcated, indurated, erythematous plaque on the central chest (Figure 1) and bilateral axillary lymphadenopathy. A biopsy specimen showed a nonspecific dermal proliferation of vascular structures (CD34+) with pleomorphic and multinucleated histiocytes (CD68+) and myofibroblasts (Figure 2). Results of immunostaining for S100, factor XIIIA, keratin, estrogen receptor, and progesterone receptor were negative. Tissue cultures were negative. View LargeDownload Figure 1. Sharply demarcated, indurated, erythematous plaque on the bilateral breasts and chest. View LargeDownload Figure 2. Dermal proliferation of vascular structures (CD34+) with pleomorphic and multinucleated histiocytes (CD68+) and myofibroblasts. A magnetic resonance image of the chest demonstrated a 5.6 × 2.0 × 3.0-cm soft-tissue mass involving the lower portion of his sternum and subcutaneous fat. Results of computed tomography–guided fine-needle aspiration showed sheets of round to oval hyperchromatic cells with scant amounts of cytoplasm. Tumor cells were strongly immunoreactive with vimentin but tested negative with CD99, CD79A, λ- and κ-light chains, actin, CD3, CD20, PTAH, cytokeratins AE1/AE3, CAM 5.2, S100, chromogranin, and synaptophysin. The pathologists concluded that the tumor represented a Ewing sarcoma–like, small, blue-cell malignant neoplasm. Findings of serum and urine protein electrophoresis and serum immunoglobulin assays were normal. A bone-marrow aspirate showed no malignancy. Further imaging demonstrated bilateral axillary lymphadenopathy and a single dense sclerotic lesion within his third lumbar vertebra. Axillary lymph node biopsy specimens revealed follicular lymphoid hyperplasia with no evidence of metastatic disease. The lumbar vertebral lesion was considered to be metastatic tumor. He received 4 cycles of chemotherapy consisting of cyclophosphamide, doxorubicin, and vincristine. This was followed by radiotherapy to the sternal tumor and third lumbar vertebral lesion. He received an additional 6 cycles of chemotherapy that consisted of ifosfamide and VP-16 for treatment of residual tumor. His skin lesion on the chest regressed 1 year after chemoradiation therapy, and the axillary lymphadenopathy resolved. Positron-emission tomography 2 years later showed no evidence of tumor. He had no evidence of disease recurrence at 6-year follow-up. Comment The term AESOP syndrome was coined by Lipsker et al.1 The clinical syndrome consists of an extensive violaceous skin patch overlying a solitary plasmacytoma of bone that is associated with regional lymphadenopathy. In the cases reported by Lipsker et al,1 histologic findings of the skin and lymph nodes were nonspecific. Treatment of the plasmacytoma resulted in regression of the skin lesion and enlarged lymph nodes. Herein, we present a similar case of a sharply demarcated, erythematous plaque over the sternum with axillary adenopathy developing over a small blue-cell sarcoma with Ewing-like features, instead of a plasmacytoma. The histologic features of the cutaneous plaque were comparable to the cases described by Lipsker et al,1 which had the hallmark of high density of small dilated vessels throughout the dermis. Regression of the primary tumor resulted in resolution of the cutaneous lesions. To our knowledge, there are only 9 patients in whom plasmacytomas presented with the AESOP syndrome.1-5 There have been no reports of other malignant tumors presenting in a similar fashion. We postulate that such a clinical syndrome is not limited to plasmacytomas but instead represents a paraneoplastic syndrome that can occur with other underlying malignant tumors. Back to top Article Information Correspondence: Dr Foo, Department of Dermatology, University of Utah, 4A330 School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132 (chong.foo@hsc.utah.edu). Conflict of Interest Disclosures: None reported. References 1. Lipsker D, Rondeau M, Massard G, Grosshans E. The AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndrome: report of 4 cases of a new syndrome revealing POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome at a curable stage. Medicine (Baltimore). 2003;82(1):51-5912544710PubMedGoogle ScholarCrossref 2. Gupta RM, Roy DC, Gupta IM, Khanna S. Extramedullary plasmacytoma IgG type I presenting as mediastinal syndrome. Br J Dis Chest. 1974;68(1):65-704819775PubMedGoogle ScholarCrossref 3. Read D, Warlow C. Peripheral neuropathy and solitary plasmacytoma. J Neurol Neurosurg Psychiatry. 1978;41(2):177-184204745PubMedGoogle ScholarCrossref 4. Feddersen RM, Burgdorf W, Foucar K, Elias L, Smith SM. Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation. J Am Acad Dermatol. 1989;21(5, pt 2):1061-10682681292PubMedGoogle ScholarCrossref 5. Weichenthal M, Stemm AV, Ramsauer J, Mensing H, Feller AC, Meigel W. POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. J Am Acad Dermatol. 1999;40(5, pt 2):808-81210321621PubMedGoogle ScholarCrossref

Journal

Archives of Dermatology – American Medical Association

Published: Dec 1, 2012

Keywords: plasmacytoma,neoplasms,skin

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