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 Table of Contents  
Year : 2020  |  Volume : 9  |  Issue : 5  |  Page : 205-209

Giant lymphangioma breast – A rare occurrence: Case report and current review

1 Department of General Surgery, Kalpana Chawla Government Medical College, Karnal, India
2 Department of General Surgery, ESIC Medical College and Hospital, NIT, Faridabad, India
3 Department of General Surgery, SGT Medical College, Gurgaon, Haryana, India

Date of Submission10-Mar-2020
Date of Decision02-Aug-2020
Date of Acceptance03-Aug-2020
Date of Web Publication12-Oct-2020

Correspondence Address:
Gulshan Kumar Garg
Kalpana Chawla Government Medical College, Karnal - 132 001, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ccij.ccij_38_20

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Cystic lymphangiomas are rare benign tumor and are due to congenital malformations of lymphatic vessels. Majority occur either at birth or up to 2 years age. These are slow-growing lesions and infiltrate into the surrounding tissues. Malignancy is quite rare in these lesions. Common sites are head and neck, axilla, and mediastinum and rarely in breast. Hereby, we report a case of a 13-year-old female who presented with a gradually increasing painless swelling in the upper outer quadrant of her left breast for the last 12 years. Magnetic resonance imaging of the breast showed multiple, irregular, hypoechoic lesions associated with irregular duct dilatation. As the treatment of choice is complete excision, the cystic mass was excised in toto and sent for histopathology, which revealed cystic lymphangioma.

Keywords: Breast, cystic lymphangioma, malignancy

How to cite this article:
Garg GK, Goyal S, Singla SL. Giant lymphangioma breast – A rare occurrence: Case report and current review. Clin Cancer Investig J 2020;9:205-9

How to cite this URL:
Garg GK, Goyal S, Singla SL. Giant lymphangioma breast – A rare occurrence: Case report and current review. Clin Cancer Investig J [serial online] 2020 [cited 2020 Oct 24];9:205-9. Available from:

  Introduction Top

Cystic lymphangiomas or cystic hygromas consist of dilated lymph channels lined by an endothelium and are uncommon benign lymphatic tumor of the breast, particularly in adults. These occur due to the following reasons: blockage of the lymphatic channels with secondary dilatation, congenital weakness of the lymphatic wall, or proliferation of lymphatic vessels, resulting in lymph node degeneration, inflammation, and fibrosis of the existing lymph channels.[1] They are generally located in the head-and-neck area, the axilla, and mediastinum, and rarely in the retroperitoneum, abdominal organs, skeleton, pancreas, scrotum, or breast. Those involving the breasts are exceptionally uncommon, and a few cases have been reported in literature.[2] Lymphangiomas are almost universally benign tumors, though rare instances of malignant transformation have been reported.[3],[4] Cystic lymphangiomas are most commonly diagnosed in young children; 50%–65% of lymphangiomas are clinically present in newborns and 90% are evident mostly by the age of 2 years.[5],[6] Although generally a pediatric issue, cystic lymphangiomas can present in adults. Adult cystic lymphangiomas are also most prevalent in the neck, but have been observed in several different organ systems.[7] Cystic lymphangiomas of the breast have also been reported, though these are exceptionally rare. Cystic lymphangiomas are always congenital, but rarely acquired lymphangiomas of breast have been reported in adults as a late sequel of mastectomy and radiation therapy.[8]

Hereby, we report a case of congenital cystic lymphangioma of the left breast in a 13-year-old girl.

  Case Report Top

A 13-year-old girl presented to the outdoor department with a complaint of a slow-growing, nontender mass in the left breast for the last 5 years. There was no associated nipple discharge or any other clinical symptoms. Physical examination revealed a nontender, slightly mobile, cystic mass in the upper, outer quadrant of the left breast measuring 20 cm × 15 cm. The overlying skin and areola were normal [Figure 1]. A clinical diagnosis of the cystic lesion of the breast was made. At first, fine-needle aspiration (FNA) cytology was performed, which was nonconclusive. Ultrasound showed a cyst in the upper and outer quadrants. Magnetic resonance imaging (MRI) was done to observe the extent of the cyst before excision [Figure 2]. Excision of the cyst was done after obtaining the consent of the patient [Figure 3]. A cystic structure measuring (20 cm × 15 cm) [Figure 4] was excised. Histopathology revealed large dilated spaces lined by a flattened endothelium growing in loose connective tissue as well as in glandular tissue of breast [Figure 5]. A final diagnosis of cystic lymphangioma of the breast was made. The diagnosis was made based on the clinical, radiological, and histopathological report.
Figure 1: Patient's image: swelling in the left breast

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Figure 2: Magnetic resonance imaging of the breast

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Figure 3: Operative part

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Figure 4: Excised cyst

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Figure 5: Histopathology report

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  Discussion Top

Cystic lymphangioma of the breast is a rare benign lymphatic tumor. It usually presents before the age of 2 years but may present later on in adolescence or adulthood, as in our case. The lymphangioma of breast, especially in adults, is an extremely unusual phenomenon. Most of the cystic lymphangiomas of the breast are mainly located in the upper outer quadrant of the breast, as in our case.[9],[10] Rarely, cystic lymphangioma may be in male breast also.[11],[12],[13] Of the known cases, most are located in the upper, outer quadrant of the breast, as this area contains ~75% of the lymphatic drainage toward the tail and axilla, as in our case[14],[15] Cystic lymphangioma is characterized by large, cyst-like spaces filled with clear lymph fluid and the spaces are lined by flat endothelial cells. Lymphangiomas can be classified into simple, cystic, and cavernous. Simple lymphangiomas consist of small, capillary-sized, thin-walled vessels with considerable connective tissue. Cystic lymphangiomas are characterized by a well-defined cyst such as spaces lined by endothelial cells and are filled with clear lymph fluid. Finally, cavernous lymphangiomas are composed of dilated lymphatic channels in a lymphatic stroma containing lymphoid aggregates.[16] Cystic lymphangioma is often associated with chromosomal abnormalities and other anatomical anomalies including Turner's syndrome, trisomy 21 and trisomy 18, as well as mosaic trisomy.[17]

The differential diagnosis of cystic lesions of the breast encompasses simple cysts, postoperative seromas, hematomas, lymphoceles, galactoceles, abscesses, hemangiomas, and, very rarely, lymphangiomas. Simple cysts are usually bilateral, and they often occur in other regions of the breast. They show a cyst with turbid yellow or greenish fluid without lining cells. Hemangiomas generally appear with thick-walled blood vessels with many red blood cells.[10],[18]

Mammography is usually nonspecific revealing fairly or ill-defined, round or lobulated densities interspersed between the normal breast parenchyma without obvious signs of micro or macrocalcification. In many cases, axillary and posterior forbidden areas may be unreachable due to macromastia.

Ultrasonography may reveal dilated noncompressible lymphatic ducts joining toward the axillary region, appearing anechoic or hypoechoic secondary to proteinaceous or hemorrhagic fluid. In early cases, cystic areas may mimic fibrocystic disease of breast, which is usually bilateral or hemangiomatous lesion, which shows vascularity on power Doppler imaging.

MRI is the modality of choice for evaluating breast lymphangioma. It helps in differentiation between benign and malignant as well as determines the extent of swelling. Breast lymphangioma appears as cystic septate masses with variably ductal dilatation and only septal enhancement, with some of the ducts/cysts showing hyperintense content on both T1-weighted and T2-weighted images due to proteinaceous or hemorrhagic contents, as seen in our case.[12],[17] Immunohistochemical investigations can distinguish between hemangioma and lymphangioma.[18]

About 31 cases of mammary cystic lymphangiomas have been reported in literature during the period of 2003–2019 [Table 1]. The reason for their late onset is not clear in all reports, and no particularly related history of pregnancy or breastfeeding was identified except in a report by de Guerké et al.[9]
Table 1: Mammary Cystic Lymphangioma (Period 2003-2019).

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As these tumors are usually present at birth, careful history taking is very significant. Delay in diagnosis is associated with increase in the size of tumor, and also there may be invasion and mixing with normal breast tissue and thus may produce the heterogeneous pattern of tumor and macromastia.[24],[25],[31],[35] The definitive diagnosis, however, can be established by core or excision biopsy. Histopathology is confirmatory for final diagnosis.[14] There was no history of breast surgery, trauma, or infection in our case. The lymphatic endothelial lining cells are strongly positive for D240 and negative for CD34 (endothelial marker for blood vessels) on immunohistochemical staining. This test was not done as this facility does not exist in our institution.[36]

FNA fluid aspirates always reveal clear, yellowish, or straw-colored fluid, with plenty of lymphocytes. However, FNA is usually not diagnostic as rarely shows cells. Therefore, surgical excision with histopathological diagnosis is the mainstay of diagnosis.[30],[37]

Lymphangiomas are treated for cosmetic and functional reasons. The choice of treatment depends on the size, depth, and location of the lesion. Undoubtedly, patient's age and general health are also important. Sclerosing agents such as steroid or bleomycin injections cause fibrosis, making surgery difficult and if used as monotherapy, there are high chances of recurrence.[38] However, for patients in whom surgery is contraindicated, guided percutaneous sclerosis can be performed.

Surgical excision is the mainstay of treatment. Ideal is wide surgical excision of the cisterns, but there may be difficulty in complete excision due to the tendency of these lesions to infiltrate into the surrounding normal breast tissues. In our case, wide excision was possible as there was no infiltration in the surrounding tissues. Incomplete excision always leads to rapid recurrence. Streptococcal lysine (OK-432), a biological response modifier, causing shrinkage of cystic spaces, can be used for small residual or recurred lymphangioma.[38]

Recurrence can occur following incomplete excision. As reported, recurrence rates of cystic lymphangiomas in pediatric groups are 100% after aspiration, 100% after injection, 40% after incomplete excision, 40% after laser treatment, and 17% after macroscopic total excision. Therefore, in mammary lesions, the goal of treatment should be preferably a complete excision to reduce the recurrence risk.[39]

  Conclusion Top

We report here a rare case of cystic lymphangioma of the breast in a 13-year-old girl. Although the incidence of this disease is extremely rare in adult breast, cystic lymphangioma should be considered in the differential diagnosis of cystic mass in the upper and outer quadrants of the breast. Early recognition of this rare disease is of great importance because complete surgical excision of small lesions and consequent cure is possible.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mirza B, Ijaz L, Saleem M, Sharif M, Sheikh A. Cystic hygroma: An overview. J Cutan Aesthet Surg 2010;3:139-44.  Back to cited text no. 1
[PUBMED]  [Full text]  
Principe DR, Rubin J, Raicu A, Hagen C. Massive adult cystic lymphangioma of the breast. J Surg Case Rep 2019;2019:rjz027.  Back to cited text no. 2
Berry JA, Wolf JS, Gray WC. Squamous cell carcinoma arising in a lymphangioma of the tongue. Otolaryngol Head Neck Surg 2002;127:458-60.  Back to cited text no. 3
Torcasio A, Veneroso S, Amabile MI, Biffoni M, Martino G, Monti M, et al. Cystic hygroma of the breast: A rare lesion. Tumori 2006;92:347-50.  Back to cited text no. 4
Hahn SY, Choi HY, Park SH, Jang J. Lymphangioma and lymphangiectasia of the breast mimicking inflammatory breast cancer. J Ultrasound Med 2011;30:863-5.  Back to cited text no. 5
Kwon SS, Kim SJ, Kim L, Kim YJ. Huge cystic lymphangioma involving the entire breast. Ann Plast Surg 2009;62:18-21.  Back to cited text no. 6
Sasi W, Schneider C, Shah R, Ruffles T, Bhagwat P, Mokbel K, et al. Recurrent cystic lymphangioma of the breast: Case report and literature review. Breast Dis 2010;31:43-7.  Back to cited text no. 7
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. 8
de Guerké L, Baron M, Dessogne P, Callonnec F, d'Anjou J. Cystic lymphangioma of the breast. Breast J 2005;11:515-6.  Back to cited text no. 9
Waqar SN, Khan H, Mekan SF, Kayani N, Raja AJ. Cystic breast lymphangioma. J Pak Med Assoc 2004;54:531-3.  Back to cited text no. 10
Ekmez F, Pirgon O, Bilgin H, Aydemir G. Cystic hygroma of the breast in a 5 year old boy presenting as a gynecomastia. Eur Rev Med Pharmacol Sci 2012;16 Suppl 4:55-7.  Back to cited text no. 11
Singh O, Gupta SS, Upadhyaya VD, Sharma SS, Lahoti BK, Mathur RK. Cystic lymphangioma of the breast in a 6-year-old boy. J Pediatr Surg 2009;44:2015-8.  Back to cited text no. 12
Gupta SS, Singh O. Cystic lymphangioma of the breast in an 8-year-old boy: Report of a case with a review of the literature. Surg Today 2011;41:1314-8.  Back to cited text no. 13
Ogun GO, Oyetunde O, Akang EE. Cavernous lymphangioma of the breast. World J Surg Oncol 2007;5:69.  Back to cited text no. 14
Chung SY, Oh KK, Kim DJ. Mammographic and sonographic findings of a breast cystic lymphangioma. J Ultrasound Med 2003;22:307-9.  Back to cited text no. 15
Rusdianto E, Murray M, Davis J, Caveny A. Adult cystic lymphangioma in the inner quadrant of the breast-Rare location for a rare disease: A case report. Int J Surg Case Rep 2016;20:123-6.  Back to cited text no. 16
Balaji R, Ramachandran K. Cystic lymphangioma of the breast: Magnetic resonance imaging features. Breast Care (Basel) 2010;5:250-2.  Back to cited text no. 17
Krainick-Strobel U, Krämer B, Walz-Mattmüller R, Kaiserling E, Röhm C, Bergmann A, et al. Massive cavernous lymphangioma of the breast and thoracic wall: Case report and literature review. Lymphology 2006;39:147-51.  Back to cited text no. 18
Yaghan RJ, Bani-Hani KE. Male breast disorders in Jordan. Disease patterns and management problems. Saudi Med J 2004;25:1877-83.  Back to cited text no. 19
Min KW, Jang SH, Na W, Jang SM, Jun YJ, Jang KS, et al. Cystic lymphangioma of the breast in an adult woman. Korean J Pathol 2008;42:244-6.  Back to cited text no. 20
Kwon SS, Sei-Joong K, Kim L, Youn-Jeong K. Huge cystic lymphangioma involving the entire breast. Ann Plast Surg 2009;62:18-21.  Back to cited text no. 21
Ogita S, Tsuto T, Nakamura K, Deguchi E, Iwai N. OK-432 therapy in 64 patients with lymphangioma. J Pediatr Surg 1994;29:784-5.  Back to cited text no. 22
Malhotra P, Bansal A, Chintamani, Saxena S. Cavernous lymphangioma of the male breast. Indian J Pathol Microbiol 2010;53:853-4.  Back to cited text no. 23
[PUBMED]  [Full text]  
Nguyen K, Karsif K, Lee S, Chorny K, Chen M. Lymphangioma in an elderly patient: An unusual cause of axillary mass. Breast J 2011;17:416-7.  Back to cited text no. 24
Hynes SO, McLaughlin R, Kerin M, Rowaiye B, Connolly CE. A unique cause of a rare disorder, unilateral macromastia due to lymphangiomatosis of the breast: A case report. Breast J 2012;18:367-70.  Back to cited text no. 25
Harbade SR, Wasadikar PP, Varudkar AS. Giant lymphangioma of the breast in an adult. J Evolution Med Dent Sci 2013;2:3257-62.  Back to cited text no. 26
Hiremath B, Binu V. Lymphangioma of the breast. BMJ Case Report 2014;(March(17)):2014.pii:bcr2014203937.  Back to cited text no. 27
Alkhalili E, Ayoubieh H, O'Brien W, Billings SD. Acquired progressive lymphangioma of the nipple. BMJ Case Rep 2014;2014:bcr2014205966.  Back to cited text no. 28
Vargas-Hernández VM, Tovar-Rodríguez JM, Moreno-Eutimio MA, Acosta-Altamirano G. Giant cystic lymphangioma breast. Report of a case with 20-year follow-up and review of the literature. Cir Cir 2014;82:81-6.  Back to cited text no. 29
Arafah M, Sweet G, Ginter PS, Hoda SA. Mammary lymphangioma. Int J Surg Pathol 2015;23:542-3.  Back to cited text no. 30
Rastogi R. Cystic lymphangioma breast: A rare tumor masquerading as carcinoma breast. Arch Can Res 2016;4:4.  Back to cited text no. 31
Almohawes E, Khoumais N, Arafah M, Pant R, Al-Bahrani R. Cystic lymphangioma of the breast: A case report in an adult woman. OMICS J Radiol 2017;6:2.  Back to cited text no. 32
Chotai N, Fok E, Chan P, Ho B. Axillary lymphangioma in an asymptomatic adult female. Breast J 2018;24:415-6.  Back to cited text no. 33
Park T, Lee HS, Jung EJ, Kim JY, Jeong CY, Ju YT, et al. Concomitant breast and axillary lymphangioma in an adult: A case report and a review of the literature. Medicine (Baltimore) 2018;97:e12946.  Back to cited text no. 34
Kook SH, Keum JS. Mammography, US, and MR findings of cystic lymphangioma of the breast: A case report. J Korean Radiol Soc 1996;35:279-81.  Back to cited text no. 35
Kaiserling E. Immunohistochemical identification of lymph vessels with D2-40 in diagnostic pathology. Pathologe 2004;25:362-74.  Back to cited text no. 36
Ko KH, Kim EK, Kang HY, Youk JH. Cavernous lymphangiomas of the breast mimicking breast cancer. J Ultrasound Med 2009;28:973-6.  Back to cited text no. 37
Okada A, Kubota A, Fukuzawa M, Imura K, Kamata S. Injection of bleomycin as a primary therapy of cystic lymphangioma. J Pediatr Surg 1992;27:440-3.  Back to cited text no. 38
Sasi W, Schneider C, Shah R, Ruffles T, Bhagwat P, Mokbel K, et al. Recurrent cystic lymphangioma of the breast: Case report and literature review. Breast Dis 2010;31:43-7.  Back to cited text no. 39


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1]


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