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E-IJD-CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 106
Acquired lymphangiectasis following surgery and radiotherapy of breast cancer


Department of Dermatology, SVS Medical College, Mahbubnagar, Andhra Pradesh, India

Date of Web Publication26-Dec-2014

Correspondence Address:
Angoori Gnaneshwar Rao
Department of Dermatology, SVS Medical College Mahbubnagar, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.147880

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   Abstract 

Acquired lymphangiectasia (AL) is a significant and rare complication of surgery and radiotherapy. We report lymphangiectasia in a 40-year-old woman who had undergone radical mastectomy and radiotherapy. After 4 years of combined therapy, she developed multiple vesicles and bullae. Skin biopsy confirmed the diagnosis of lymphangiectasia. The case is unique as it is not associated with lymphedema, which is a usual accompaniment of lymphangiectasia following surgery and radiotherapy. AL is usually asymptomatic, but trauma may cause recurrent cellulitis. Treatment modalities include electrodessication, surgical excision, sclerotherapy and carbon dioxide laser ablation.


Keywords: Acquired lymphagiectasia, lymphedema, radiation, surgery


How to cite this article:
Rao AG. Acquired lymphangiectasis following surgery and radiotherapy of breast cancer. Indian J Dermatol 2015;60:106

How to cite this URL:
Rao AG. Acquired lymphangiectasis following surgery and radiotherapy of breast cancer. Indian J Dermatol [serial online] 2015 [cited 2020 Feb 20];60:106. Available from: http://www.e-ijd.org/text.asp?2015/60/1/106/147880

What was known?
Acquired lymphangiectasia is known to occur following surgery and/or radiotherapy for carcinoma breast. It is usually associated with lymphedema.



   Introduction Top


Acquired lymphangiectasia (AL) is a dilation of lymphatic vessels, which results after damage caused to the previously normal lymphatics. The damage can primarily be due to surgical intervention alone, irradiation alone, or by surgery and irradiation combined or secondary to scarring. In 1956, Plotnick and Richfield first described it as a complication of radical mastectomy. [1] AL most commonly occurs in adults as a late sequel of surgery and radiation therapy (RT). It has also been associated with metastatic lymph node invasion and obstruction. AL clinically manifests as translucent vesicles in a chronic lymphedematous area several years after surgery with or without RT. [2]


   Case Report Top


A 40-year-old woman was reported with multiple vesicles and bullae on the front and right side of the chest for 2 years. She was diagnosed as a case of infiltrating duct cell carcinoma of the right breast in the year 2006 for which she underwent radical mastectomy followed by the radiotherapy. She noticed the vesicular eruption 4 years after surgery and radiotherapy. Cutaneous examination revealed the absence of the right breast (post mastectomy). Multiple grouped vesicles and bullae were spread over the right anterior, lateral wall of the chest, and the hypochondrium. A Y-shaped scar was noticed in the middle of the right mammary region (mastectomy scar) with no evidence of lymphedema underneath [Figure 1]. Few of the vesicles and bullae were purple in color; pedunculated, and hypertrophic [Figure 2]. The clinical findings prompted us to diagnose AL. However, lymphangioma circumscriptum (LC), lymphangiomatous cutaneous metastases, and angiosarcoma were considered in the differential diagnosis. Histopathological examination of skin biopsy revealed, numerous dilated lymphatics in the superficial and papillary dermis lined by flattened endothelial cells, with mild hyperkeratosis consistent with diagnosis of lymphangiectasia [Figure 3] and [Figure 4]. Immunohistochemistry with podoplanin (specific marker for lymphatic endothelium) could not be carried out due to paucity of funds. She was managed with electrodessication.
Figure 1: Multiple grouped vesicles and bullae on the right mammary region and lateral wall of chest and right hypochondrium

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Figure 2: Purple and translucent vesicles and hypertrophic pedunculated bullae

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Figure 3: Histopathology skin showing numerous dilated lymphatics in the superficial and papillary dermis (H and E, ×10)

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Figure 4: Histopathology skin showing dilated lymphatics in the superficial and papillary dermis lined by flattened endothelial cells with mild hyperkeratosis (H and E, ×40)

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   Discussion Top


AL represents acquired vesicular dilation of lymphatic channels secondary to an external cause. It has been reported in the literature with an increased frequency in the past 20 years due to increase in surgical excision, surgery and RT for certain malignancies of breast and cervix. [3],[4],[5] AL was also reported as a sequel to the treatment of myxoid chondrosarcoma and bronchial carcinoid. [6],[7] It characteristically presents with the vesicles and bullae. Some of these may turn purple due to the presence of red cells, which arise from hemangiolymphatic connections. Occasionally, they may become pedunculated with the hyperkeratotic and verrucous surface resembling wart. The index case is presented with similar features. Coexisting lymphedema is a usual association in most patients of AL. However, there was no associated lymphedema in the reported case. It is usually asymptomatic, but trauma may provide portal of entry for infection giving rise to recurrent cellulitis. [3] Scarring from scrofuloderma, scleroderma, and cirrhosis are also known to cause AL without lymphedema. [8],[9],[10] Chiyomaru and Nishigor in a retrospective study of 73 cases of AL following treatment for malignant neoplasm notified external genitalia as the most frequent site. Furthermore, they reported that the combination of surgery and irradiation (77%) was the most frequent preceding therapy, followed by surgery alone (18%) and irradiation alone (5%). In addition, it was found that the mean interval from completion of therapy to the development of AL was shorter after combination therapy (5.8 years) than after surgery (12.2 years) or irradiation (11.8 years) alone. [11] Stewart et al., have also reported AL in their study of 8 patients of carcinoma cervix following surgery and/or radiotherapy. [12] Gnanraj et al., have also notified two cases of AL following surgery and radiotherapy for carcinoma cervix. [13] AL has been reported to occur 3-26 years after the surgery, [12],[13],[14],[15],[16] [Table 1]. The index case developed AL 4 years after surgery and radiotherapy, which is relatively short, compared to the other studies in the literature.
Table 1: Interval between therapy and development of lymphangiectasia in various case studies

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The pathogenesis of AL is unclear; however, surgical damage most likely causes fibrosis and lymphatic obstruction at the base of the reticular dermis and accumulation of lymph fluid in the dermal lymphatics with resultant increased pressure. This can then lead to dilation of the superficial lymphatic channels with subsequent vesicle formation. RT also contributes in its pathogenesis because the main site of injury of RT is at the junction of the fat and reticular dermis., [5],[17] RT itself is known to cause lymphangiectasia by causing lymphangiogenesis in the exposed area during the 1 st year of therapy. Recently, it was found that there was an increased number of podoplanin-positive lymphatic vessels and CD68-positive histiocytes in cancer patients treated with the radiotherapy. [18] AL patients with the chronic lymphedema following mastectomy and/or radiotherapy may develop angiosarcoma. [19] These angiosarcomas are characterized immunohistochemically by expression and amplification of myelocytomatosis (MYC) gene, which differentiates post radiation angiosarcomas from benign vascular proliferations following radiotherapy. [20] In addition, immunohistochemical staining for MYC not only helps in the diagnosis but also helps in mapping the neoplasm.

AL is indistinguishable both clinically and histopathologically from LC. Histologically, both reveal ectatic lymphatic spaces in the papillary dermis, which are lined by normal or flattened endothelial cells. The overlying epidermis may show hyperkeratosis and acanthosis. Although, there are no histological criteria to differentiate LC from AL some believe that the subcutaneous muscle-coated cisternae characteristic of LC are absent in AL.

Various therapeutic modalities have been advocated for the treatment of lymphangiectasis. These include surgical excision, cryotherapy, electrodesiccation, sclerotherapy, and laser therapy. [3],[4],[5] Various types of lasers have been used for the treatment including the carbondioxide, [21] argon and tunable dye. [22] The combination of radiofrequency ablation with sclerotherapy was found to be simple, efficacious and cost-effective with a long-term satisfactory results. [23] Gianelli and Rockley found electrodessication superior to the other treatment modalities. [4] Each therapeutic option is effective, but recurrences are common and may be complicated by scarring.

 
   References Top

1.
Plotnick H, Richfield D. Tuberous lymphangiectatic varices secondary to radical mastectomy. Arch Dermatol 1956;74:466-8.  Back to cited text no. 1
    
2.
Leshin B, Whitaker DC, Foucar E. Lymphangioma circumscriptum following mastectomy and radiation therapy. J Am Acad Dermatol 1986;15:1117-9.  Back to cited text no. 2
    
3.
Celis AV, Gaughf CN, Sangueza OP, Gourdin FW. Acquired lymphangiectasis. South Med J 1999;92:69-72.  Back to cited text no. 3
    
4.
Giannelli V, Rockley PF. Acquired lymphangiectasis following mastectomy and radiation therapy: Report of a case and review of the literature. Cutis 1996;58:276-8.  Back to cited text no. 4
    
5.
Mallett RB, Curley GK, Mortimer PS. Acquired lymphangioma: Report of four cases and a discussion of the pathogenesis. Br J Dermatol 1992;126:380-2.  Back to cited text no. 5
    
6.
Moon SE, Youn JI, Lee YS. Acquired cutaneous lymphangiectasia. Br J Dermatol 1993;129:193-5.  Back to cited text no. 6
    
7.
Ziv R, Schewach-Millet M, Trau H. Lymphangiectasia. A complication of thoracotomy for bronchial carcinoid. Int J Dermatol 1988;27:123.  Back to cited text no. 7
    
8.
Di Leonardo M, Jacoby RA. Acquired cutaneous lymphangiectasias secondary to scarring from scrofuloderma. J Am Acad Dermatol 1986;14:688-90.  Back to cited text no. 8
    
9.
Tuffanelli DL. Letter: Lymphangiectasis due to scleroderma. Arch Dermatol 1975;111:1216.  Back to cited text no. 9
    
10.
García-Doval I, de la Torre C, Losada A, Ocampo C, Rodríguez T, Cruces MJ. Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites. J Eur Acad Dermatol Venereol 1999;13:109-12.  Back to cited text no. 10
    
11.
Chiyomaru K, Nishigori C. Acquired lymphangiectasia associated with treatment for preceding malignant neoplasm: A retrospective series of 73 Japanese patients. Arch Dermatol 2009;145:841-2.  Back to cited text no. 11
    
12.
Stewart CJ, Chan T, Platten M. Acquired lymphangiectasia ('lymphangioma circumscriptum') of the vulva: A report of eight cases. Pathology 2009;41:448-53.  Back to cited text no. 12
    
13.
Gnanaraj P, Revathy V, Venugopal V, Tamilchelvan D, Rajagopalan V. Secondary lymphangioma of vulva: A report of two cases. Indian J Dermatol 2012;57:149-51.  Back to cited text no. 13
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14.
Tasdelen I, Gokgoz S, Paksoy E, Yerci O, Cetintas SK, Demiray M, et al. Acquired lymphangiectasis after breast conservation treatment for breast cancer: Report of a case. Dermatol Online J 2004;10:9.  Back to cited text no. 14
    
15.
Kaya TI, Kokturk A, Polat A, Tursen U, Ikizoglu G. A case of cutaneous lymphangiectasis secondary to breast cancer treatment. Int J Dermatol 2001;40:760-1.  Back to cited text no. 15
    
16.
Jappe U, Zimmermann T, Kahle B, Petzoldt D. Lymphangioma circumscriptum of the vulva following surgical and radiological therapy of cervical cancer. Sex Transm Dis 2002;29:533-5.  Back to cited text no. 16
    
17.
Mortimer PS, Simmonds RH, Rezvani M, Robbins ME, Ryan TJ, Hopewell JW. Time-related changes in lymphatic clearance in pig skin after a single dose of 18 Gy of X rays. Br J Radiol 1991;64:1140-6.  Back to cited text no. 17
    
18.
Jackowski S, Janusch M, Fiedler E, Marsch WC, Ulbrich EJ, Gaisbauer G, et al. Radiogenic lymphangiogenesis in the skin. Am J Pathol 2007;171:338-48.  Back to cited text no. 18
    
19.
Rodríguez-Bujaldón A, Vázquez-Bayo MC, Galán-Gutiérrez M, Jiménez-Puya R, Vélez García-Nieto A, Moreno-Giménez JC, et al . Angiosarcoma in chronic lymphedema. Actas Dermosifiliogr 2006;97:525-8.  Back to cited text no. 19
    
20.
Manner J, Radlwimmer B, Hohenberger P, Mössinger K, Küffer S, Sauer C, et al. MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema. Am J Pathol 2010;176:34-9.  Back to cited text no. 20
    
21.
Bailin PL, Kantor GR, Wheeland RG. Carbon dioxide laser vaporization of lymphangioma circumscriptum. J Am Acad Dermatol 1986;14:257-62.  Back to cited text no. 21
    
22.
Weingold DH, White PF, Burton CS. Treatment of lymphangioma circumscriptum with tunable dye laser. Cutis 1990;45:365-6.  Back to cited text no. 22
    
23.
Khunger N. Combination technique of radiofrequency ablation with sclerotherapy in acquired lymphangiectasis of the vulva. J Cutan Aesthet Surg 2009;2:33-5.  Back to cited text no. 23
[PUBMED]  Medknow Journal  

What is new?
AL without lymphedema is known to occur following scarring in scrofuloderma, scleroderma, and cirrhosis. The absence of lymphedema in the reported case of AL without an underlying disease is novel and interesting. The short time interval between the treatment (surgery and radiotherapy) and the development of AL is noteworthy in the reported case.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

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    Abstract
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