- Email: [email protected]
Angiosarcoma in an obese woman with worsening lymphedema after weight-loss and skin-reduction surgeries
J AM ACAD DERMATOL
448 Letters
AUGUST 2011
Emily A. Moosbrugger, MD,a and Brian B. Adams, MD, MPHa,b Department of Dermatology, University of Cincinnati,a and Section of Dermatology, Veterans Affairs Medical Center,b Cincinnati, Ohio Funding sources: None. Conflicts of interest: None declared. Reprint requests: Brian B. Adams, MD, MPH, PO Box 670592, Cincinnati, OH 45267 E-mail: [email protected]
REFERENCES 1. Magina S, Lisboa C, Leal V, Palmares J, Mesquita-Guimar~aes J. Dermatological and ophthalmological sequels in toxic epidermal necrolysis. Dermatology 2003;207:33-6. 2. Oplatek A, Brown K, Sen S, Halerz M, Supple K, Gamelli RL. Long-term follow-up of patients treated for toxic epidermal necrolysis. J Burn Care Res 2006;27:26-33. 3. Shoji T, Cockerell CJ, Koff AB, Bhawan J. Eruptive melanocytic nevi after Stevens-Johnson syndrome. J Am Acad Dermatol 1997;37:337-9. 4. Schirren CG, Jansen T, Lindner A, Kind P, Plewig G. Diffuse sebaceous gland hyperplasia: a case report and an immunohistochemical study with cytokeratins. Am J Dermpathol 1996;18:296-301. 5. Marini M, Saponaro A, Remorino L, Lynch P, Magarinos G. Eruptive lesions in a patient with bone marrow transplantation. Int J Dermatol 2001;40:133-5. 6. Azevedo RS, Almeida OP, Netto JN, Miranda AM, Santos TC, Della Coletta R, et al. Comparative clinicopathological study of intraoral sebaceous hyperplasia and sebaceous adenoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;107:100-4. ~ ate Y, Soler E, Hernandez-Machın B, Montene7. Guillermo N, Pen gro T, Borrego L. Bilateral areolar sebaceous hyperplasia in a female. Int J Dermatol 2008;47:1214-5. 8. Ortiz-Rey JA, Martın-Jimenez A, Alvarez C, De La Fuente A. Sebaceous gland hyperplasia of the vulva. Obstet Gynecol 2002;99:919-21. doi:10.1016/j.jaad.2010.02.035
Angiosarcoma in an obese woman with worsening lymphedema after weight-loss and skin-reduction surgeries To the Editor: Cutaneous angiosarcomas are rare, aggressive tumors that comprise approximately 2% of all soft tissue sarcomas. Their overall prognosis is very poor, with a 5-year survival rate ranging from 10% to 35%.1 Epithelioid angiosarcoma is a rare variant of cutaneous angiosarcoma that can have equally aggressive behavior.2 The development of cutaneous angiosarcoma may be a rare, but significant, possible side effect of the lymphedema associated with both obesity and weight loss or skin reduction surgery.
Fig 1. Right calf with 17- 3 8-cm violaceous ulcerated, multifocal tumor.
Fig 2. Large nodular collections of mitotically active epithelioid cells filled the superficial and deep dermis. Atypical epithelioid cells lined irregularly jagged vascular spaces (inset).
A 59-year-old woman presented to Dermatology with a large, rapidly enlarging multifocal tumor on her right calf (Fig 1). Her medical history was notable for obesity, chronic mild bilateral lower extremity edema, and gastric bypass surgery 4 years before presentation. Subsequently, she underwent abdominoplasty and thigh reduction surgery to remove excess skin and developed significant worsening bilateral lower extremity edema. A biopsy specimen of the right calf tumor demonstrated large nodular collections of mitotically active epithelioid cells throughout the dermis. The atypical epithelioid cells focally lined irregular vascular spaces in a redundant fashion (Fig 2). By immunohistochemistry, the cells stained moderately with antibodies to Ulex europaeus, factor VIII, CD31, and CD34. The atypical cells did not stain with antibodies against CD3, AE1/3, Cam5.2, S-100, CD45RB, CD20, or CK20. These histologic findings were consistent with an epithelioid angiosarcoma. Initial staging did not demonstrate metastatic disease. Two months after an above-knee amputation, metastases developed in the scalp, mediastinum, lung, and several lymph nodes. Treatment with
J AM ACAD DERMATOL VOLUME 65, NUMBER 2
paclitaxel was initiated, but several months later a refractory malignant pleural effusion developed and the patient died soon thereafter. The development of angiosarcoma in areas of chronic lymphedema is well documented. Reports have described its occurrence in patients with lymphedema secondary to lymph node dissection, filarial infection, congenital lymphedema, and chronic idiopathic lymphedema.2 There have been 3 reported cases of angiosarcoma (one with epithelioid and kaposiform features that did not stain with antibody to HHV-8) developing in the abdominal wall pannus of morbidly obese patients ranging in age from 35 to 81 years old.3 The patients had chronic lymphedematous changes secondary to their obesity. Two of the patients developed metastatic disease and died within 10 months of diagnosis; the third patient had no follow-up beyond 7 months. Additionally, a fourth case documented the development of angiosarcoma in an obese 64-year-old man with abdominal wall lymphedema 7 years after abdominal dermolipectomy and subsequent thoracoplasty with bilateral resection of the subaxillary fat tissue and bilateral subcutaneous mastectomy. The patient died 1 year later from metastatic disease.4 The prevalence of obesity, bariatric surgery, and skin reduction surgery continues to rise. Lymphedema associated with these surgeries may contribute to the risk of developing cutaneous angiosarcoma, a rare, yet aggressive tumor. Maria R. Robinson, MD, Kord S. Honda, MD, and Jeremy S. Bordeaux, MD, MPH Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio Funding sources: None. Conflicts of interest: None declared. Reprints not available from the authors. Correspondence to: Jeremy S. Bordeaux, MD, MPH, Department of Dermatology, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44016. E-mail: [email protected] REFERENCES 1. Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma. A report of 67 patients and a review of the literature. Cancer 1996;77:2400-6. 2. Requena L, Sangueza OP. Cutaneous vascular proliferations, part III: malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. J Am Acad Dermatol 1998;38(2 pt 1):143-75.
Letters 449
3. Shehan JM, Ahmed I. Angiosarcoma arising in a lymphedematous abdominal pannus with histologic features reminiscent of Kaposi’s sarcoma: report of a case and review of the literature. Int J Dermatol 2006;45:499-503. 4. Aguiar Bujanda D, Camacho Galan R, Bastida Inarrea J, Aquiar Morales J, Conde Martel A, Rivero Suarez P, et al. Angiosarcoma of the abdominal wall after dermolipectomy in a morbidly obese man. A rare form of presentation of Stewart-Treves syndrome. Eur J Dermatol 2006;16:290-2. doi:10.1016/j.jaad.2010.03.004
Genital edema in childhood: Harbinger of Crohn’s disease? To the Editor: A 13-year-old boy presented with a 7-month history of a slightly progressive asymptomatic swelling of the penis and scrotum. He was previously treated with 3 different oral antibiotics without effect. He had a history of constipation, but had no other gastrointestinal complaints. Physical examination revealed an edematous, slightly erythematous, and indurated skin of the penis and scrotum (Fig 1). In the right groin, enlarged lymph nodes were palpable. His weight and height were at the 5th percentile for his age. Skin biopsy revealed vascular and lymphatic ectasia with non-caseating granulomas and giant cells located around lymphatic vessels (Fig 2). Ultrasonography of the genitals and groins showed a subcutaneous swelling of penis and scrotum. At this point our main differential diagnosis consisted of granulomatous lymphangitis, sarcoidosis, and mycobacterial infections. Chest x-ray was normal. Auramine and periodic acideSchiff stainings, culture, and polymerase chain reaction (PCR) for Mycobacterium tuberculosis and atypical mycobacteria on skin biopsy yielded negative findings. Laboratory evaluation, consisting of complete blood cell count, erythrocyte sedimentation rate, albumin, total protein, angiotensin-converting enzyme level, lysozyme and Borrelia serology, revealed no abnormalities. Our patient was referred to the Department of Pediatrics for further evaluation. On gastroscopy, multiple aphthous lesions of the stomach and duodenum were detected. Histologic examination of these lesions showed limited chronic inflammation, but was not specific for Crohn’s disease. Colonoscopy revealed no abnormalities. Video capsule endoscopy was performed, which showed ulcerations in the ileum. On magnetic resonance imaging (MRI), thickening of the terminal ileum wall was observed. Although histologic study was not conclusive, the results from skin biopsy, colonoscopy, video capsule endoscopy, and MRI taken together did support a
448 Letters
AUGUST 2011
Emily A. Moosbrugger, MD,a and Brian B. Adams, MD, MPHa,b Department of Dermatology, University of Cincinnati,a and Section of Dermatology, Veterans Affairs Medical Center,b Cincinnati, Ohio Funding sources: None. Conflicts of interest: None declared. Reprint requests: Brian B. Adams, MD, MPH, PO Box 670592, Cincinnati, OH 45267 E-mail: [email protected]
REFERENCES 1. Magina S, Lisboa C, Leal V, Palmares J, Mesquita-Guimar~aes J. Dermatological and ophthalmological sequels in toxic epidermal necrolysis. Dermatology 2003;207:33-6. 2. Oplatek A, Brown K, Sen S, Halerz M, Supple K, Gamelli RL. Long-term follow-up of patients treated for toxic epidermal necrolysis. J Burn Care Res 2006;27:26-33. 3. Shoji T, Cockerell CJ, Koff AB, Bhawan J. Eruptive melanocytic nevi after Stevens-Johnson syndrome. J Am Acad Dermatol 1997;37:337-9. 4. Schirren CG, Jansen T, Lindner A, Kind P, Plewig G. Diffuse sebaceous gland hyperplasia: a case report and an immunohistochemical study with cytokeratins. Am J Dermpathol 1996;18:296-301. 5. Marini M, Saponaro A, Remorino L, Lynch P, Magarinos G. Eruptive lesions in a patient with bone marrow transplantation. Int J Dermatol 2001;40:133-5. 6. Azevedo RS, Almeida OP, Netto JN, Miranda AM, Santos TC, Della Coletta R, et al. Comparative clinicopathological study of intraoral sebaceous hyperplasia and sebaceous adenoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;107:100-4. ~ ate Y, Soler E, Hernandez-Machın B, Montene7. Guillermo N, Pen gro T, Borrego L. Bilateral areolar sebaceous hyperplasia in a female. Int J Dermatol 2008;47:1214-5. 8. Ortiz-Rey JA, Martın-Jimenez A, Alvarez C, De La Fuente A. Sebaceous gland hyperplasia of the vulva. Obstet Gynecol 2002;99:919-21. doi:10.1016/j.jaad.2010.02.035
Angiosarcoma in an obese woman with worsening lymphedema after weight-loss and skin-reduction surgeries To the Editor: Cutaneous angiosarcomas are rare, aggressive tumors that comprise approximately 2% of all soft tissue sarcomas. Their overall prognosis is very poor, with a 5-year survival rate ranging from 10% to 35%.1 Epithelioid angiosarcoma is a rare variant of cutaneous angiosarcoma that can have equally aggressive behavior.2 The development of cutaneous angiosarcoma may be a rare, but significant, possible side effect of the lymphedema associated with both obesity and weight loss or skin reduction surgery.
Fig 1. Right calf with 17- 3 8-cm violaceous ulcerated, multifocal tumor.
Fig 2. Large nodular collections of mitotically active epithelioid cells filled the superficial and deep dermis. Atypical epithelioid cells lined irregularly jagged vascular spaces (inset).
A 59-year-old woman presented to Dermatology with a large, rapidly enlarging multifocal tumor on her right calf (Fig 1). Her medical history was notable for obesity, chronic mild bilateral lower extremity edema, and gastric bypass surgery 4 years before presentation. Subsequently, she underwent abdominoplasty and thigh reduction surgery to remove excess skin and developed significant worsening bilateral lower extremity edema. A biopsy specimen of the right calf tumor demonstrated large nodular collections of mitotically active epithelioid cells throughout the dermis. The atypical epithelioid cells focally lined irregular vascular spaces in a redundant fashion (Fig 2). By immunohistochemistry, the cells stained moderately with antibodies to Ulex europaeus, factor VIII, CD31, and CD34. The atypical cells did not stain with antibodies against CD3, AE1/3, Cam5.2, S-100, CD45RB, CD20, or CK20. These histologic findings were consistent with an epithelioid angiosarcoma. Initial staging did not demonstrate metastatic disease. Two months after an above-knee amputation, metastases developed in the scalp, mediastinum, lung, and several lymph nodes. Treatment with
J AM ACAD DERMATOL VOLUME 65, NUMBER 2
paclitaxel was initiated, but several months later a refractory malignant pleural effusion developed and the patient died soon thereafter. The development of angiosarcoma in areas of chronic lymphedema is well documented. Reports have described its occurrence in patients with lymphedema secondary to lymph node dissection, filarial infection, congenital lymphedema, and chronic idiopathic lymphedema.2 There have been 3 reported cases of angiosarcoma (one with epithelioid and kaposiform features that did not stain with antibody to HHV-8) developing in the abdominal wall pannus of morbidly obese patients ranging in age from 35 to 81 years old.3 The patients had chronic lymphedematous changes secondary to their obesity. Two of the patients developed metastatic disease and died within 10 months of diagnosis; the third patient had no follow-up beyond 7 months. Additionally, a fourth case documented the development of angiosarcoma in an obese 64-year-old man with abdominal wall lymphedema 7 years after abdominal dermolipectomy and subsequent thoracoplasty with bilateral resection of the subaxillary fat tissue and bilateral subcutaneous mastectomy. The patient died 1 year later from metastatic disease.4 The prevalence of obesity, bariatric surgery, and skin reduction surgery continues to rise. Lymphedema associated with these surgeries may contribute to the risk of developing cutaneous angiosarcoma, a rare, yet aggressive tumor. Maria R. Robinson, MD, Kord S. Honda, MD, and Jeremy S. Bordeaux, MD, MPH Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio Funding sources: None. Conflicts of interest: None declared. Reprints not available from the authors. Correspondence to: Jeremy S. Bordeaux, MD, MPH, Department of Dermatology, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44016. E-mail: [email protected] REFERENCES 1. Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma. A report of 67 patients and a review of the literature. Cancer 1996;77:2400-6. 2. Requena L, Sangueza OP. Cutaneous vascular proliferations, part III: malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. J Am Acad Dermatol 1998;38(2 pt 1):143-75.
Letters 449
3. Shehan JM, Ahmed I. Angiosarcoma arising in a lymphedematous abdominal pannus with histologic features reminiscent of Kaposi’s sarcoma: report of a case and review of the literature. Int J Dermatol 2006;45:499-503. 4. Aguiar Bujanda D, Camacho Galan R, Bastida Inarrea J, Aquiar Morales J, Conde Martel A, Rivero Suarez P, et al. Angiosarcoma of the abdominal wall after dermolipectomy in a morbidly obese man. A rare form of presentation of Stewart-Treves syndrome. Eur J Dermatol 2006;16:290-2. doi:10.1016/j.jaad.2010.03.004
Genital edema in childhood: Harbinger of Crohn’s disease? To the Editor: A 13-year-old boy presented with a 7-month history of a slightly progressive asymptomatic swelling of the penis and scrotum. He was previously treated with 3 different oral antibiotics without effect. He had a history of constipation, but had no other gastrointestinal complaints. Physical examination revealed an edematous, slightly erythematous, and indurated skin of the penis and scrotum (Fig 1). In the right groin, enlarged lymph nodes were palpable. His weight and height were at the 5th percentile for his age. Skin biopsy revealed vascular and lymphatic ectasia with non-caseating granulomas and giant cells located around lymphatic vessels (Fig 2). Ultrasonography of the genitals and groins showed a subcutaneous swelling of penis and scrotum. At this point our main differential diagnosis consisted of granulomatous lymphangitis, sarcoidosis, and mycobacterial infections. Chest x-ray was normal. Auramine and periodic acideSchiff stainings, culture, and polymerase chain reaction (PCR) for Mycobacterium tuberculosis and atypical mycobacteria on skin biopsy yielded negative findings. Laboratory evaluation, consisting of complete blood cell count, erythrocyte sedimentation rate, albumin, total protein, angiotensin-converting enzyme level, lysozyme and Borrelia serology, revealed no abnormalities. Our patient was referred to the Department of Pediatrics for further evaluation. On gastroscopy, multiple aphthous lesions of the stomach and duodenum were detected. Histologic examination of these lesions showed limited chronic inflammation, but was not specific for Crohn’s disease. Colonoscopy revealed no abnormalities. Video capsule endoscopy was performed, which showed ulcerations in the ileum. On magnetic resonance imaging (MRI), thickening of the terminal ileum wall was observed. Although histologic study was not conclusive, the results from skin biopsy, colonoscopy, video capsule endoscopy, and MRI taken together did support a