# Bilateral Giant Juvenile Fibroadenomas of Breasts:A Case Report

Authors: D. B. Nikumbh; S. R. Desai; P. S. Madan; N. J. Patil; J. V. Wader
Journal: Pathology Research International
 (2011)

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## Abstract

Juvenile fibroadenoma constitutes only 4% of the total fibroadenomas. The  incidence of giant juvenile fibroadenomas is found to be only 0.5% of all the fibroadenomas. Bilateral giant  juvenile  fibroadenomas are extremely rare, and only four cases have been reported  in  the literature. To the best of our knowledge, we are presenting the fifth case of bilateral giant juvenile fibroadenomas in a 12-year-old prepubertal girl. The diagnosis  was  made on fine-needle aspiration cytology which was confirmed on histopathology. In this paper, we present this  rare case  to illustrate the diagnosis and management of this tumour and to emphasize that  these tumours are almost always benign and should be treated with breast-conserving surgery to provide a healthy physical and social life to the  patient.


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## Body

## 1. Introduction

Breast masses are uncommon in childhood. The majority of them are related to inflammation (infection or abscess) [1] or benign tumors as fibroadenomas [2]. Juvenile fibroadenoma is a rare clinical entity and forms 4% of thetotal fibroadenomas, and giant juvenile fibroadenoma constitutes only 0.5% of all fibroadenomas [3, 4]. Bilateral giant juvenile fibroadenomas are extremely rare in prepubertal girls. To the best of our knowledge, only four case reports are available in the English literature [3, 5–7]. Stanford School of Medicine clearly defined both the entities of juvenile and giant fibroadenoma [8]. Our case satisfied both these definitions; therefore, it is a rare case of bilateral giant juvenile fibroadenomas in a twelve-year-old prepubertal female child. The provisional diagnosis of the patient was made on fine-needle aspiration cytology which was subsequently confirmed on histopathology.


## 2. Case Report

A  12-year-old  premenarche  girl  presented  with  bilateral,  rapidly  enlarging  breast  lumps  for  three  months  (Figure1). There was history of dull ache in the breasts. There was no family history.  History  of   trauma,  nipple  discharge,  fever,  anorexia,  or  weight  loss  was  absent. On  local  examination   bilateral   slightly  tender,  huge,  well-circumscribed  masses  in  both  the  breasts  were  seen,  which  were  firm  in  consistency.  The  overlying  skin  was  tense  and  shiny  with  prominent  superficial  veins.  The right breast lump measured  15×12 cms and left breast lump measured  17×15 cms.  On  both  the  sides, the  lumps  were  not  fixed  to  underlying  structures.  There was no discharge from   the nipple, and axillary lymphadenopathy was absent.  Routine hematological and biochemical examinations were within normal limits. Chest-X ray was normal.  Ultrasonography  was  carried  out  which showed   heterogenous  parenchymal  pattern  suggestive  of   bilateral  fibroadenoma. The patient was  subjected  to  fine-needle  aspiration  cytology  (FNAC)  of   bilateral  breast  lumps  which  revealed  cellular  smears   comprising  of   many  branching   monolayer  sheets  along  with  myoepithelial  cells  (Figure 2).Figure 1
Gross appearance of bilateral breasts tumors.Figure 2
FNAC from breast lumps showed many branching sheets of epithelial cells with many bare nuclei and myxoid stromal fragments in background. (H &E stain X100).Background showed bare nuclei and myxoid stromal fragments.  Based  on  the  cytological  findings,  diagnosis  of   benign  proliferative  lesion  without  atypia  suggestive  of  bilateral  fibroadenomas  was  given. The patient  underwent  total  excision  of  bilateral  breast  lumps  conserving  the  normal  breast  tissue,  nipple, and areola  by  the  plastic  surgeon. The  two  resected  specimens  were  sent  for  histopathological  study  in  the department of pathology.  The right-sided excised lump measured15×12×6.5 cms and weighed 800 gms. The left-sided excised lump measured 17×15×5 cms and weighed 950 gms. Overlying skin was unremarkable. External  surface  was  bosselated,  and  both  the lumps  were  well  circumscribed  and  encapsulated (Figure 3).  Cut  sections  from  both  the  lumps  showed  multiple  gray  white  nodules  showing  myxoid, gray  white  appearance  along  with  occasional  slit-like  spaces  and  tiny  cysts (Figure 4).Figure 3
Gross appearance of bilateral lumpectomy specimens.Figure 4
Cut surface of both the specimens showed multiple gray white nodules with myxoid appearance and occasional slit-like spaces.


## 3. Light  Microscopic  Findings

Hematoxylin- and  eosin-stained  sections  revealed  well-encapsulated  tumors  with  hyperplasia  of   both  epithelial  and  stromal  components.  Epithelial  component  showed  tubular  or  tufted  pattern  of  epithelial  hyperplasia  with  characteristic  tufts  of  cells  protruding  into  the  lumina (Figure5).   The  glands  were  lined  by  bilayered  epithelium  at  places  showing  stratification. Most  of  the  areas  in  both  the  tumors  showed  a pericanalicular  pattern  of  fibroadenoma.  Stroma  was  cellular  and  showed  myxoid  change (Figure 6). Though   stromal   cellularity  was  increased, there  was  no  leaf-like  growth  pattern  and   focal  periductal  concentrate  of  cells  which  was  the  characteristic  of  phyllodes  tumor.  There was occasional mitosis (0-1/hpf) and absence of   cytological atypia.  The  final  histopathological  diagnosis  of   Bilateral  Giant  Juvenile  Fibroadenomas  of   breasts  was  given. The  patient  is  on  regular  followup  and  doing  well  at  six-month  followup.Figure 5
Light microscopy showed well-encapsulated tumor with hyperplasia of both epithelial and stromal components. (H&E stain X100).Figure 6
Light microscopy revealed many glands lined by bilayered epithelium at places showed stratification and cellular stoma with myxoid change (H&E Stain X400).


## 4. Discussion

According  to  Stanford  School  of   Medicine,  juvenile  fibroadenoma  of  the  breast  is  defined  as circumscribed,  often   large,  breast  mass  usually  occurring  in  adolescent  females  with  stromal  and  epithelial    hypercellularity   but  lacking  the  leaf-like  growth  pattern  of  phyllodes  tumors [8]. Diagnostic criteria for juvenile fibroadenoma are (1) circumscribed and rarely multiple; (2) biphasic   stromal  and  epithelial  process  in  which  pericanalicular  pattern  is  most  common and lacks  leaf-like  growth  pattern  in  uniformly  hypercellular  stroma. Fibrotic  areas  may  be  present; (3) lack  of  atypical  features  in  stroma-like  periductal  increase  in  cellularity, stromal overgrowth, cytologic  atypia, and mitotic  rate   >3/hpf; (4)  frequent  epithelial  and  myoepithelial  hyperplasia; (5)  most  patients’ age  is 10–20  years  with a mean  age  of  15  years. Juvenile fibroadenomas may be multiple [8].Giant  fibroadenoma  is  defined  as  a  tumor  >500 gms  or  disproportionally  large  compared  to the rest  of   the   breast. It  is  more  frequently  seen  in  young  and  black  patients.  Giant fibroadenoma   may be either adult type or juvenile fibroadenoma [8, 9].Giant  juvenile  fibroadenoma  simultaneously  occurring  in  both  the  breasts  is  very  rare.  Four  case  reports  are  available  in  the  English  literature [3, 5–7].    Giant  juvenile  fibroadenoma  is  an uncommon  tumor presenting  in  adolescent  females  and  the  exact  etiology  is  not  known. Hormonal  influences  are  thought  to  be  contributing  factors [9]. Excessive  estrogen  stimulation  and/or receptor  sensitivity   or  reducedlevels  of  estrogen  antagonist  during  puberty  have  been  implicated  in  pathogenesis [9, 10].It  is  necessary  to  exclude  the  close  differentials  of  juvenile  fibroadenoma  which  are  benign  low-grade  phyllodes  tumor,  virginal  hypertrophy, and  other  rare  differentials  such  as  lipoma,  hamartoma, breast  abscess,  macrocyst,  adenocarcinoma, and  pseudoangiomatous  stomal  hyperplasia, as  the  treatment  modalities  and  the  prognosis  differ  quite  significantly  in  these  various conditions.  Some  of  the  lesions were treated  by  mastectomy, but  some  lesions  may  require  only  local  excision,  aspiration, or   conservative  management [10–12].Giant  juvenile   fibroadenoma  is  a  benign  tumor, and  total  excision  of the  lump  with  conservation  of  nipple  and  areola  is  the  optimal treatment [10–12].Benign  low-grade  phyllodes  tumor  occurs  in  the  older (>40 years)  age  group  and has no  racial  predisposition. Generally, they  present  as  a  solitary  mass  confined  to  unilateral  breast, and  bilateral  involvement  is  rarely  seen [10].  In  low-grade  phyllodes  tumor,  prominent  leaf-like  architecture  is  seen   due  to  stromal  cellularity  and, there  is  a characteristic  stromal  condensation  around  the  ducts, and  it  infiltrates the surrounding  breast  tissue  with  mitotic  figures  <4/hpf.  In  high-grade  phyllodes  tumor, stromal  overgrowth  with  atypia  and  atypical  mitotic  figures (<10/hpf)  are  seen.  It  is  treated  by  wide  excision  with  a  margin  of  normal  tissue  or  mastectomy [10, 11, 13, 14].In  juvenile  breast  hypertrophy,  rapid  and  distressing  enlargement  of  one  or  both  breasts occurs, which  is  often  asymmetrical  and  occasionally    in  an  adolescent  female. Histological  examination   shows  abundant  connective  tissue  and  duct  proliferation,  frequently  with  epithelial  hyperplasia  but  little  or  no  lobule  formation.  It is treated by reduction mammoplasty [10, 12, 14].Giant lipoma can cause unilateral breast hypertrophy.  Soft, mobile mass can be felt on palpation. Lipoma  consists  of  encapsulated  nodules  of  mature  adipose  tissue  on  histology [10, 12, 14].Breast  abscesses  developing  during  puberty  cause  sudden  and  rapid  growth  in  the  breast.  Pain, fluctuation, and erythema are present. Histological  examination  reveals  focal  collection  of  polymorphs  with  necrotic  material  in  the  lobules. Hamartomas  can  be  easily  suspected  on  imaging  with  their  multilobular  structures.  Microscopically,  these  lesions  are  composed  of  an  admixture  of  ducts,  lobules,  fibrous  stroma,  and  adipose  tissue  in  varying  proportions [13, 14]. Pseudoangiomatous  stromal  hyperplasia  reveals  complex  interanastomosing  spaces, some  of  which  have  spindle-shaped  stromal  cells  at  their  margins  simulating  endothelial cells [10, 12, 14]. FNAC and biopsy easily rule out these conditions.It  is  essential  to  know  that  giant  juvenile  fibroadenoma  may  recur  after  complete  excision, and  the  chance  of  recurrence  becomes  less  after the third  decade [13]. In  view  of   bilateral  giant  juvenile  fibroadenomas, possibility  of  Carneys  complex  should  be  considered, which  constitutes  multiple  myxoid  fibroadenomas, endocrine  hyperactivity,  cardiac  myxoma,  cutaneous  hyperpigmentation,  and  other  abnormalities [14].In  our  case,  other  components  of  Carneys  complex  were  absent   with the lack  of  family  history   except for multiple  fibroadenomas.Isolated  case  reports  ofunilateral   juvenile  fibroadenoma  and  multiple  giant  fibroadenoma  in  single  breast   were  available [9, 12, 15, 16].  In  the  literature, only  four  case  reports  of  bilateral  giant  juvenile  fibroadenomas  were  reported [3, 5–7]. The last case was reported in 2009 [7]. To  conclude,  we  present  an  extremely  rare  case  of   bilateral  giant  juvenile  fibroadenomas  of  breasts.  The  case  was  diagnosed  on  FNAC  and  subsequently  confirmed  on  histopathology. The  patient  is  treated  by the removal  of   both  the  fibroadenomas   conserving  the  breast  tissue.  The patient is doing well with   regular followup.



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*Source: 482046-2011-05-30.xml*