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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 113-116

Giant juvenile fibroadenoma in a young female-A diagnostic dilemma


Department of Pediatric Surgery, ABVIMS and Dr. RML Hospital, New Delhi, India

Date of Submission24-Apr-2021
Date of Decision29-Apr-2021
Date of Acceptance02-May-2021
Date of Web Publication31-May-2021

Correspondence Address:
Dr. Pinaki Ranjan Debnath
Flat No-Cgv 193, V-Tower, Dlf Capital Greens, Moti Nagar, New Delhi - 110 015
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_91_21

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  Abstract 

Background: Fibroadenoma is the most common breast lesion among pediatric female population. A giant fibroadenoma is rare, characterized by a rapidly growing tumor, with a mass >5 cm diameter in greatest dimension and/or weighing more than 500 gm. Phyllodes tumor range from benign to malignant. Both have similar presentation. Clinical Description: An 11-year-old girl presented with rapidly progressive, painless increase in the size of the left breast over 2 months. Local examination revealed a firm nontender mass involving the upper inner and outer quadrants of the left breast along with the retro-areolar region. The mass was mobile and measured approximately 11 cm × 10 cm. The overlying skin was normal, although with dilated veins and free from underlying mass. The clinical phenotype was suggestive a phyllodes tumor in view of rapid progression and large size. However, the ultrasonogram and fine-needle aspiration cytology favored a fibroadenoma. Management: It was decided to perform a “Nipple-areolar complex sparing lumpectomy” with deferment of reconstructive surgery. Adequate lump excision was achieved while maintaining proper cosmesis. The histopathological findings of the excision biopsy confirmed the final diagnosis of fibroadenoma. Conclusion: A large breast mass in a pediatric/adolescent girl poses a diagnostic dilemma to the treating surgeon. Proper evaluation is needed to differentiate between several benign breast masses from malignant ones. The definitive diagnosis is made histologically. Total excision of the lump with conservation of nipple and areola is indicated to make a definitive diagnosis and to relieve the compression of the normal breast tissue.

Keywords: Breast, giant juvenile fibroadenoma, phyllodes tumor


How to cite this article:
Gautam V, Khanna C, Meena AK, Debnath PR. Giant juvenile fibroadenoma in a young female-A diagnostic dilemma. Indian Pediatr Case Rep 2021;1:113-6

How to cite this URL:
Gautam V, Khanna C, Meena AK, Debnath PR. Giant juvenile fibroadenoma in a young female-A diagnostic dilemma. Indian Pediatr Case Rep [serial online] 2021 [cited 2021 Jun 19];1:113-6. Available from: http://www.ipcares.org/text.asp?2021/1/2/113/317373

Breast development is a part of the normal physical changes in an adolescent female. Any asymmetry of size, abnormality, or mass in the breast is an issue of concern for affected individuals and their families.[1] Most breast masses in the pediatric age group are benign in nature. Fibroadenoma is the most common benign tumor found in the breast. It usually affects individuals younger than 30 years and is common in African Americans. It is referred to as a juvenile fibroadenoma if it manifests between 10 and 18 years of age. A giant fibroadenoma is a rare variant (constituting 0.5% of all fibroadenomas) that is characterized by a rapidly growing tumor, with a mass >5 cm diameter in the greatest dimension and/or weighing more than 500 gm.[2] It is important to differentiate giant juvenile fibroadenoma from other breast mass lesions with same clinical presentation, namely phyllodes tumor and virginal hypertrophy as the management options differ. Fibroadenoma being a benign condition can be observed in some cases or treated by surgical excision while phyllodes tumor can be either benign or malignant and treatment requires excision with negative margins.[3] On the other hand, virginal breast hypertrophy is a rare benign disease, characterized by rapid and excessive growth of one or both breasts during the peripubertal period. Symptomatic treatment is applied as a first step and the reconstruction process may be delayed until the postpubertal period.

We present this case report because of the rarity of the condition, the challenges it poses to the treating surgeon in terms of establishing diagnosis and performing surgical excision with or without breast reconstruction.


  Clinical Description Top


An 11-year-old prepubertal girl presented with a month history of asymmetrical enlargement of the left breast. The sudden increase in size of the left breast in comparison to the other was initially mistaken as normal breast growth. Localized pain, discomfort, redness, or nipple discharge was absent. There was no history of preceding trauma, fever, anorexia, or weight loss. There was no significant family history of similar complaints in any female members of the family. The girl had never been exposed to radiation or taken any estrogen supplementation other hormones or drugs in the past.

General physical examination revealed stable vital parameters, age-appropriate body mass index, and sexual maturity rating (Tanner Stage III) in the unaffected breast. There was no pallor, icterus, or significant cervical, axillary or inguinal lymphadenopathy. On local examination, a firm nontender mass was felt involving the upper inner and outer quadrants of the left breast along with the retro-areolar region. The mass was mobile and measured approximately 11 cm × 10 cm [Figure 1]. The overlying skin was normal and free from underlying mass, although dilated veins were present over the swelling. There was no nipple discharge. The right breast was normal. There were no significant findings on the systemic examination.
Figure 1: Large mass occupying upper part of left breast

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On the basis of the seemingly benign clinical presentation, two close differential diagnoses were kept, phyllodes tumor (in view of the short history, rapid progression, and large size) and giant fibroadenoma (in view of the age). Confirmatory investigations (localized imaging and histopathological examination) were planned to differentiate between the two.

Management and Outcome

Routine hematological investigations did not reveal any evidence of acute or chronic inflammation. Biochemical tests were normal. Ultrasonography of the left breast showed a large homogeneous lesion involving almost the entire breast, indicative of a fibroadenoma. Fine-needle aspiration cytology (FNAC) smears were moderately cellular, consisting of benign ductal epithelial cells arranged in large cohesive sheets, tissue fragments, cohesive clusters and focal cribriform pattern. Interspersed myoepithelial cells and occasional stromal fragments were seen, also suggestive of a fibroadenoma.

The patient and her parents were counselled regarding the need of lumpectomy and possible disfigurement of the breast postoperatively. It was also explained that the risk of a malignant phyllodes tumor would only be confirmed after postoperative tissue biopsy. A plastic surgery consult was taken for considering postsurgical breast reconstruction. Delayed breast reconstruction was advised in view of better outcomes after the completion of pubertal breast growth. Often, normal breast development takes care of the discrepancy in size that occurs directly postoperatively, and reconstruction is not required at all. Thus, conservative surgical excision was planned by “Nipple-areolar complex sparing lumpectomy,” in order that the compression of the normal breast tissue by the abnormal tissue would be relieved. Intraoperatively, the mass was delivered intact, i.e., the lump was excised leaving the nipple-areolar complex in situ. The lumpectomy specimen appeared grossly homogeneous, was grayish white in color, and 10 cm × 10 cm × 5 cm in size, with a skin flap of 8 cm × 3.5 cm with grossly unremarkable skin surface [Figure 2]a and [Figure 2]b. The histopathological examination revealed circumscribed well-encapsulated tumor comprising of biphasic epithelial and stromal proliferation [Figure 3]. The epithelial component shows glands with pericanalicular patterns and mild epithelial hyperplasia with preserved myoepithelial layer. No significant cytological atypia noted in epithelial component. The stromal component showed mild cellularity and mild anisonucleosis. The final diagnosis was that of ajuvenile giant fibroadenoma. The postoperative course was uneventful, with drain removal on the 3rd postoperative day and discharge by the 4th day. When the patient was followed up after a week, the wound and nipple areolar complex were healthy. There was no discharge from the wound or necrosis of the skin flap or nipple areolar complex.
Figure 2: (a) preoperative photograph with marking of incision site (b) excised well encapsulated mass with excised skin

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Figure 3: Photograph taken after closure of incision

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  Discussion Top


Giant juvenile fibroadenomas are rubbery, mobile, and nontender masses that are composed of epithelium and/or stroma of the terminal lobule of the breast. The most common site is the upper outer quadrant of the breast. They are multiple in 10%–15% cases and exhibit bilateral involvement in 10%. Although etiology is still unknown, hormonal factors have been implicated. Some major factors postulated are excessive estrogen stimulation, increased estrogen receptor sensitivity, and/or decreased estrogen antagonist sensitivity.[3]

It is difficult to differentiate a giant juvenile fibroadenoma from other that share the same presentation, i.e., phyllodes tumors and virginal hypertrophy. A giant fibroadenoma disfigures the breast and may affect the growth of normal breast tissue due to a direct pressure effect but carries no malignant potential. In contrast, the nature of a phyllodes tumor ranges from being benign to being locally malignant. Virginal hypertrophy occurs due to estrogenic stimulation, resulting in unequal growth of the breast buds and leading to asymmetrical enlargement. Other clinical features that need to be factored in while arriving at a clinical diagnosis are age, duration, and size: Phyllodes occur in older individuals, display rapid growth and are large (3 cm and larger)[4] and bossellated whereas fibroadenomas are seen in younger patients, demonstrate slower progression and are usually small, except for giant fibroadenomas.

Tissue biopsy is critical in establishing the diagnosis in large lesions. Histologically, phyllodes tumors differ from giant fibroadenomas by the presence of leaf-like structures and stromal cell atypia.[5] Virginal hypertrophy is characterized by abundant connective tissue with duct proliferation and lack of lobule formation. Isolated FNAC cannot differentiate between a phyllodes tumor and giant fibroadenoma[6] and require core needle biopsy for greater accuracy. However, due to the psychological and emotional effects of core needle biopsy in young patients, excisional biopsy is preferred as a diagnostic cum therapeutic method.[7],[8]

The goal of treatment is complete excision of the tumor, preservation of the areola and nipple, and achievement of symmetrical breasts.[7],[8] Reconstructive methods are usually needed after resection of large breast lesions to attain bilateral symmetry. However, according to a systematic review, reconstructive surgery is not commonly done in giant fibroadenomas.[7],[8] In our case, conservative surgery was performed as the preoperative diagnosis was still unclear clinical suspicion being a phyllodes tumor, while FNAC favoring a fibroadenoma.

There are three basic principles for breast reconstruction: Preserving all the normal breast parenchyma; adjusting the skin envelope; and positioning the nipple-areola complex so that it is symmetrical with the opposite breast.[9] The extremely large size of tumor led us to anticipate the possibility of requiring reconstructive surgery, but we managed adequate closure with sparing of nipple areolar complex in our patient, despite the size [Figure 4]. The decision of a nipple-areolar sparing lumpectomy was taken preoperatively so that adequate cosmesis could be achieved, if reconstruction was needed. Thus, we managed to follow all the three principles and achieved adequate lump excision while maintaining proper cosmesis. The long-time prognosis is good as the lesion is benign.
Figure 4: Circumscribed encapsulated nodule composed of admixture of epithelial and stromal elements in near equal proportions. (H&E x 40)

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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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
AlGhamdi S, Ali AG, Ali SN, et al. Giant juvenile fibroadenoma of breast in adolescent girls. JPS Case Rep 2018;28:33-6.  Back to cited text no. 1
    
2.
Jayasinghe Y, Simmons PS. Fibroadenomas in adolescence. Curr Opin Obstet Gynecol 2009;21:402-6.  Back to cited text no. 2
    
3.
Çelik MF, Dural AC, Ünsal MG, et al. Giant juvenile fibroadenoma. Turk J Surg. 2015;31:96-8.  Back to cited text no. 3
    
4.
Leraas HJ, Rosenberger LH, Ren Y, et al. Pediatric phyllodes tumors: A review of the National Cancer Data Base and adherence to NCCN guidelines for phyllodes tumor treatment. J Pediatr Surg 2018;53:1123-8.  Back to cited text no. 4
    
5.
Makhija D, Shah H, Bothra J, et al. An adolescent with a phyllodes tumor: A case report and review. Int J Pediatr Adolesc Med 2016;3:180-3.  Back to cited text no. 5
    
6.
Makar GS, Makar M, Ghobrial J, et al. Malignant phyllodes tumor in an adolescent female: A rare case report and review of the literature. Case Rep Oncol Med 2020;2020:1989452.  Back to cited text no. 6
    
7.
Sosin M, Pulcrano M, Feldman ED, et al. Giant juvenile fibroadenoma: A systematic review with diagnostic and treatment recommendations. Gland Surg 2015;4:312-21.  Back to cited text no. 7
    
8.
Thuruthiyath N, Das PC, Avabratha KS, et al. Giant fibroadenoma of breast in an adolescent girl. Oman Med J 2012;27:314-5.  Back to cited text no. 8
    
9.
Chang DS, McGrath MH. Management of benign tumors of the adolescent breast. Plast Reconstr Surg 2007;120:13e-19e.  Back to cited text no. 9
    


    Figures

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



 

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