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Cystic hygroma of the breast
European
Journal of Radiology, 11 ( 1990) 2 15-2 17
215
Elsevier
EURRAD
00102
Cystic hygroma of the breast Rafael Salvador ‘, Manuel From the Departments
Salvador ‘, Dolores Miranda ‘, Miguel Rul12 and Dolores of ‘Radiodiagnosis.
‘Surger_v and 3Pathology, Universidad Autbnoma de Barcelona,
(Received 28 May 1990; accepted
Lopez 3
Spain
after revision 28 June 1990)
Key words: Breast, cystic hygroma; Breast, lymphangioma;
Breast, ultrasound
study
Introduction Cystic hygromas are unusual benign tumors that originate as a developmental anomaly of the lymphatic system, and part of the family of the lymphangiomas. They are supposed to be the result of a congenital blockage of regional lymphatic drainage, or of sequestered lymphatic sacs [l-2]. Their most common appearance is a cervical mass in the young child, but the diagnosis of a cystic hygroma should be considered when a lump in the axillary tail of the breast in a young adult has to be evaluated.
Case report A 19-year-old girl attended our Institution for a painful lump in her breast. Physical examination demonstrated a large non-adherent hard mass in the axillary tail of the right breast. Mammography (Fig. 1) showed an extremely dense, expansive mass in this location, with a sharp contour. An ultrasound scan (Fig. 2) revealed a 10 x 9 cm heterogeneous mass containing big anechogenic areas and multiple hyperechogenic linear septa. A line-needle aspiration puncture of the mass was performed, obtaining 100 cc of a reddish fluid. The cytologic study showed a high population of lymphocytes. After surgical excision of the mass, the hystologic study revealed a large hard mass 6.5 x 5 x 2 cm, with multiple inner cystic cavities, covered by a thin layer of flattened cells, and with muscle fascicles and lympho-
Address for reprints: Rafael 08007-Barcelona, Spain. 0720-048X/90/$03.50
0
Salvador,
1990 Elsevier
M.D.,
C/Valencia
Science Publishers
223,
Fig. 1. Mammography. Right axillary projection. An extreme1 dense, radiographically benign mass is seen in the axillary tail.
B.V. (Biomedical
Division)
216
Fig. 2. Sonography of the mass. Big sonolucent mass with internal linear septa and distal acoustic enhancement.
cytes in the walls (Fig. 3). It was diagnosed as a cystic hygroma of the third type, according to the classilication of Landing and Farber [ 31. Discussion Lymphangiomas are unusual congenital malformations of the lymphatic system, existing of single or multiple cavities covered by endothelium and containing fluid [4]. The biggest congenital types, often diagnosed prenatally, have been associated to Turner’s syndrome [ 51. They are usually diagnosed in infancy: 50% shortly after birth, and 90% are generally evident before the second year of life. There is no sex predominance [2,6,7], but, in some series [l] the right-sided lymphangiomas are more common than the left-sided. The normal appearance is an asymptomatic soft tissue mass, that can either grow slowly or very fast after an internal bleeding, inflammation or trauma [2,6], or without any obvious reason [ 11. They are normally located posterior or laterally in the neck (75x), mediastinal(3-10%) and in the axilla (20%), as in our case. Unusual locations are the mediastinum, the retroperitoneum, the abdominal organs, the skeletal system and the scrotum [2,7].
Fig. 3. Hystologic section of cystic lymphangioma (H + E x 400). Lymphocyte aggregates with muscle layers in the wall of the cavities.
Some axillary forms have been described in pediatric populations, but we have not found any previous description of lymphangioma presenting as a breast mass in an adult woman. The differential diagnosis of a cystic heterogeneous mass should include branchial cleft cyst, abscess and hematoma; however the clinical picture is usually characteristic in these cases [2,8,9]. Other benign tumor conditions, such as the highly echogenic hemangiomas, should also be included [ lo]. Sheth [2], in a recent study on cystic hygromas in children, concluded that the sonographic findings in a multiloculated cystic mass with a sharp border and inner solid areas should strongly suggest the diagnosis of cystic hygroma. In our case the correct diagnosis was not assessed, in spite of
217
its typical sonographic features, due to the age of the patient and its unusual location. References 1 &hefter RP, Olsen KD, Gaffey TA. Cervical lymphangioma in the adult. Otolaryngol Head Neck Surg 1985; 93: 65-69. 2 Sheth S, Nussbaum AR, Hutchins GM, Sanders RC. Cystic hygromas in children: sonographic-pathologic correlation. Radiology 1987; 162: 821-824. 3 Landing BH, Farber S. Tumors ofthe cardiovascular system. In: Atlas of tumor pathology, Section 3, Fascicle 7. Washington, DC: Armed Forces Institute of Pathology, 1956; l-138. 4 Phillips E, McGaham, P. Intrauterine fetal cystic hygromas: sonographic detection. AJR 1981; 136: 799-802.
5 Gembruch U, Hansmann M, Bald R, Zerres K, Schwanitz G, Fddisch HJ. Prenatal diagnosis and management in fetuses with cystic hygromata colli. Eur J Obstet Gynecol Reprod Biol 1988; 29: 241-255. 6 Emery PJ, Bailey CM, Evans JNG. Hygroma of the head and neck. J Laryngol Otol 1984; 98: 613-619. 7 Singh S, Baboo ML, Pathak LC. Cystic lymphangioma in children: Report of 32 cases including lesions at rare sites. Surgery 1971; 69: 947-951. 8 Badami JP, Athey PA. Sonography in the diagnosis of branchial cysts. AJR 1981; 137: 1245-1248. 9 Sherman NH, Rosenberg HK. Heyman S, Templeton J. Ultrasound evaluation of neck masses in children. J Ultrasound Med 1985; 4: 127-134. 10 Kraus R, Han BK, Babcock D, Oestreich A. Sonography of neck masses in children. AJR 1986; 146: 609-613.
Journal of Radiology, 11 ( 1990) 2 15-2 17
215
Elsevier
EURRAD
00102
Cystic hygroma of the breast Rafael Salvador ‘, Manuel From the Departments
Salvador ‘, Dolores Miranda ‘, Miguel Rul12 and Dolores of ‘Radiodiagnosis.
‘Surger_v and 3Pathology, Universidad Autbnoma de Barcelona,
(Received 28 May 1990; accepted
Lopez 3
Spain
after revision 28 June 1990)
Key words: Breast, cystic hygroma; Breast, lymphangioma;
Breast, ultrasound
study
Introduction Cystic hygromas are unusual benign tumors that originate as a developmental anomaly of the lymphatic system, and part of the family of the lymphangiomas. They are supposed to be the result of a congenital blockage of regional lymphatic drainage, or of sequestered lymphatic sacs [l-2]. Their most common appearance is a cervical mass in the young child, but the diagnosis of a cystic hygroma should be considered when a lump in the axillary tail of the breast in a young adult has to be evaluated.
Case report A 19-year-old girl attended our Institution for a painful lump in her breast. Physical examination demonstrated a large non-adherent hard mass in the axillary tail of the right breast. Mammography (Fig. 1) showed an extremely dense, expansive mass in this location, with a sharp contour. An ultrasound scan (Fig. 2) revealed a 10 x 9 cm heterogeneous mass containing big anechogenic areas and multiple hyperechogenic linear septa. A line-needle aspiration puncture of the mass was performed, obtaining 100 cc of a reddish fluid. The cytologic study showed a high population of lymphocytes. After surgical excision of the mass, the hystologic study revealed a large hard mass 6.5 x 5 x 2 cm, with multiple inner cystic cavities, covered by a thin layer of flattened cells, and with muscle fascicles and lympho-
Address for reprints: Rafael 08007-Barcelona, Spain. 0720-048X/90/$03.50
0
Salvador,
1990 Elsevier
M.D.,
C/Valencia
Science Publishers
223,
Fig. 1. Mammography. Right axillary projection. An extreme1 dense, radiographically benign mass is seen in the axillary tail.
B.V. (Biomedical
Division)
216
Fig. 2. Sonography of the mass. Big sonolucent mass with internal linear septa and distal acoustic enhancement.
cytes in the walls (Fig. 3). It was diagnosed as a cystic hygroma of the third type, according to the classilication of Landing and Farber [ 31. Discussion Lymphangiomas are unusual congenital malformations of the lymphatic system, existing of single or multiple cavities covered by endothelium and containing fluid [4]. The biggest congenital types, often diagnosed prenatally, have been associated to Turner’s syndrome [ 51. They are usually diagnosed in infancy: 50% shortly after birth, and 90% are generally evident before the second year of life. There is no sex predominance [2,6,7], but, in some series [l] the right-sided lymphangiomas are more common than the left-sided. The normal appearance is an asymptomatic soft tissue mass, that can either grow slowly or very fast after an internal bleeding, inflammation or trauma [2,6], or without any obvious reason [ 11. They are normally located posterior or laterally in the neck (75x), mediastinal(3-10%) and in the axilla (20%), as in our case. Unusual locations are the mediastinum, the retroperitoneum, the abdominal organs, the skeletal system and the scrotum [2,7].
Fig. 3. Hystologic section of cystic lymphangioma (H + E x 400). Lymphocyte aggregates with muscle layers in the wall of the cavities.
Some axillary forms have been described in pediatric populations, but we have not found any previous description of lymphangioma presenting as a breast mass in an adult woman. The differential diagnosis of a cystic heterogeneous mass should include branchial cleft cyst, abscess and hematoma; however the clinical picture is usually characteristic in these cases [2,8,9]. Other benign tumor conditions, such as the highly echogenic hemangiomas, should also be included [ lo]. Sheth [2], in a recent study on cystic hygromas in children, concluded that the sonographic findings in a multiloculated cystic mass with a sharp border and inner solid areas should strongly suggest the diagnosis of cystic hygroma. In our case the correct diagnosis was not assessed, in spite of
217
its typical sonographic features, due to the age of the patient and its unusual location. References 1 &hefter RP, Olsen KD, Gaffey TA. Cervical lymphangioma in the adult. Otolaryngol Head Neck Surg 1985; 93: 65-69. 2 Sheth S, Nussbaum AR, Hutchins GM, Sanders RC. Cystic hygromas in children: sonographic-pathologic correlation. Radiology 1987; 162: 821-824. 3 Landing BH, Farber S. Tumors ofthe cardiovascular system. In: Atlas of tumor pathology, Section 3, Fascicle 7. Washington, DC: Armed Forces Institute of Pathology, 1956; l-138. 4 Phillips E, McGaham, P. Intrauterine fetal cystic hygromas: sonographic detection. AJR 1981; 136: 799-802.
5 Gembruch U, Hansmann M, Bald R, Zerres K, Schwanitz G, Fddisch HJ. Prenatal diagnosis and management in fetuses with cystic hygromata colli. Eur J Obstet Gynecol Reprod Biol 1988; 29: 241-255. 6 Emery PJ, Bailey CM, Evans JNG. Hygroma of the head and neck. J Laryngol Otol 1984; 98: 613-619. 7 Singh S, Baboo ML, Pathak LC. Cystic lymphangioma in children: Report of 32 cases including lesions at rare sites. Surgery 1971; 69: 947-951. 8 Badami JP, Athey PA. Sonography in the diagnosis of branchial cysts. AJR 1981; 137: 1245-1248. 9 Sherman NH, Rosenberg HK. Heyman S, Templeton J. Ultrasound evaluation of neck masses in children. J Ultrasound Med 1985; 4: 127-134. 10 Kraus R, Han BK, Babcock D, Oestreich A. Sonography of neck masses in children. AJR 1986; 146: 609-613.