Case Recap
This 12-year old girl presented to her mother's gynecologist with a new right breast mass that grew rapidly over a 2 month period. She noted that the skin of the right breast was tight and shiny, and the breast was becoming painful. There was also occasionally a small amount of white discharge from the right nipple. Menarche was six months prior to the onset of the mass, and there is a history of pre-menopausal breast cancer in a maternal aunt.
Physical exam revealed a very thin young girl with marked asymmetry between the right and left breasts. The left breast was entirely normal, without any palpable abnormalities. The right breast had an ill-defined large mass occupying the entire central portion of the breast, and on exam the mass was just beneath the skin of the nipple-areolar complex. It was fixed to the underlying breast tissue.
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Figure 1
Patient and the outline of the mass within the breast
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Figure 2:
Removal of mass through inframammary incision.
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Figure 3:
Post-operative photo
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Expert Commentary: Four Views
PEDIATRIC AND ADOLESCENT GYNECOLOGY
Patricia Simmons, MD
The presence of a breast mass in an adolescent provokes not only concern and anxiety for the patient and her family, but for her physician. Most education and experience physicians have with breast masses comes from the adult population, where the risk of a malignant neoplasm is substantially higher than for the young. Adenocarcinoma is almost exclusively a disease of the adult breast. Malignancies of the adolescent breast, while uncommon, are more likely to be solid tumors more common to this age group, including rhabdomyosarcoma, lymphoma, and neuroblastoma. The most common large breast mass in the adolescent is a benign fibroadenoma. When a mass is large and/or rapidly growing, phyllodes tumor is also a consideration, and though most are benign, they can be malignant.
Evaluation of an adolescent with a breast mass should recognize relative differences in frequency of various neoplasms and development-specific differences in the role of imaging modalities. The density of the adolescent breast makes mammography an insensitive modality. Ultrasound is preferred, because it is more sensitive and helps differentiate fibroadenoma from a more worrisome neoplasm. When malignancy is suspected, because of other worrisome features in the patient's clinical presentation (history of malignancy, history of chest radiation therapy, constitutional symptoms, lymphadenopathy, hepatosplenomegaly, other masses, characteristics of the breast mass itself), then specific imaging with CT or MR is useful. Of note, other risk factors for breast adenocarcinoma (family history, BRCA gene, chronic anovulation, etc..) would not yet manifest as breast cancer in this young of a patient. The most important factor in determining her risk for malignancy is the presence of a history or findings suggestive of an adolescent malignancy.
What we know about this patient is that she is an otherwise healthy 12 year old with a large, rapidly growing breast mass. In the absence of other symptoms, risk factors, and findings on physical examination, the most likely diagnosis is fibroadenoma. Phyllodes tumor of the breast is also a consideration, particularly since the mass is large and rapidly growing. To help better narrow the differential diagnosis before surgery, an ultrasound would be helpful. If the sonogram is consistent with fibroadenoma or phyllodes tumor, the next step is an excisional biopsy. I would only perform a fine needle aspirate or core needle biopsy if the clinical presentation and/or ultrasound supported the possibility of another type of malignant tumor for which therapy would not include excision of the mass.
Prognosis and follow up would depend on whether this tumor was found on excisional biopsy to be a benign fibroadenoma, benign phalloides, or had likely in phyllodes.
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PATHOLOGY
Ann Thor, MD and Adeboye O. Osunkoya, MD
Giant juvenile fibroadenoma is a benign, unifocal and often rapidly growing painless tumor that presents during adolescence. GJF most frequently occurs in African American women and has been attributed to a localized hypersensitivity to gonadal hormones.1 GJF must be distinguished from other pathologic entities. The differential diagnosis includes a usual fibroadenoma, other benign lesions, cystosarcoma phyllodes, carcinoma, or sarcoma. Giant juvenile fibroadenoma shows stromal and epithelial hyperplasia with stromal cellularity and ductal epithelium in a peri or intracanalicular pattern. In some of these tumors the ductal cells manifest significant hyperplasia, with micropapillary, solid or cribriform patterns that can mimic in situ carcinoma.2 Mitotic figures and significant cytologic atypism are infrequent.
Cystosarcoma phylloides (CP) is a rare breast tumor that may occur at any age, is typically unifocal, nonpainful, and quickly growing. Cystosarcoma phyllodes demonstrate a leaflike microarchitecture, stromal atypia and mitotic figures.3 Although the term 'giant fibroadenoma' has been used interchangeably with the term 'benign phyllodes tumor' it is important to subdivide these rare tumors more accurately into GJF and CP categories. Recent molecular studies have demonstrated that the giant juvenile fibroadenoma is a polyclonal cellular process, whereas cytosarcoma phyllodes is a monoclonal neoplasm.4
Giant juvenile fibroadenoma can be distinguished from asymmetric breast hypertrophy and hamartomas by their clinical presentation and lack of mammary lobules on microscopic examination. Juvenile breast hypertrophy can be unilateral or bilateral, and presents as a diffuse breast enlargement without nodularity or a discrete mass. The treatment for breast hypertrophy is reduction mammaplasty.5 Hamartomas of the breast are rare and are often underdiagnosed. They present as a painless breast lump, an enlarged or slowly enlarging breast.6 Hamartomas are rarely as firm as fibroadenomas and they are typically slow growing. The average age at presentation for harmartoma is 45 years. On physical examination they lesions are well-circumscribed, mobile and are soft-to-firm in consistency - similar to the surrounding and contralateral breast.
Primary breast cancers of the breast are exceedingly rare in adolescent women, although GFT must be distinguished from carcinoma or sarcoma. The microscopic features of GFT are at first glance reminiscent of ductal carcinoma in situ (DCIS). DCIS typically occurs in older women, is a precursor lesion for invasive cancer and is characterized by specific architectural patterns, more marked cytologic atypia, frequent tumor necrosis and a lack of stromal cellularity.7 Because DCIS is so rare in adolescence, the diagnosis of DCIS in this age group should prompt slide review and diagnostic confirmation by an expert breast pathologist prior to treatment. Primary sarcoma of the breast is also very rare and typically occurs in older women. Sarcomas of the breast are derived from specialized periductular or perilobular stromal cells. Both carcinomas and sarcomas are typically monophasic and lack the biphasic stromal and epithelial cell populations of GFT.8
In summary, GFT is a rare polyclonal cellular response that results from a localized hypersensitivity to gonadal hormones. The resulting tumorlike mass is typically fast growing and occurs in young adolescent women, particularly African Americans. GFT should be distinguished from other benign or malignant entities by core or open biopsy, followed by histopathologic examination. Although rare, the rapid growth of GFT can cause significant patient and physician concern. Pathology examination will allow for a definitive diagnosis to be rendered, facilitate treatment and follow up planning.
References
1. Dehner LP, Hill DA, Deschryver K. Pathology of the breast in children, adolescents, and young adults. Semin Diagn Pathol. 1999 Aug;16(3):235-47.
2. Mies C, Rosen PP. Juvenile fibroadenoma with atypical epithelial hyperplasia. Am J Surg Pathol. 1987 Mar;11(3):184-90.
3. Rajan PB, Cranor ML, Rosen PP. Cystosarcoma phylloides in adolescent girls
and young women: a study of 45 patients. Am J Surg Pathol. 1998 Jan;22(1):64-9.
4. Noguchi S, Aihara T, Motomura K, Inaji H, Imaoka S, Koyama H, Tanaka H. Demonstration of polyclonal origin of giant fibroadenoma of the breast. Virchows Arch. 1995;427(3):343-7.
5. Wechselberger G, Pulzl P, Pichler M, Schoeller T, Piza-Katzer H. Juvenile gigantomastia treated by reduction mammoplasty. Am J Surg. 2004 Sep;188(3):333-4.
6. Weinzweig N, Botts J, Marcus E. Giant hamartoma of the breast. Plast Reconstr Surg. 2001 Apr 15;107(5):1216-20.
7. Wells WA, Carney PA, Eliassen MS, Grove MR, Tosteson AN. Pathologists' agreement with experts and reproducibility of breast ductal carcinomain-situ classification schemes. Am J Surg Pathol. 2000 May;24(5):651-9.
8. Callery CD, Rosen PP, Kinne DW. Sarcoma of the breast. A study of 32 patients with reappraisal of classification and therapy. Ann Surg. 1985 Apr;201(4):527-32
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ONCOPLASTIC SURGERY
Gail Lebovic, MD
The case presented illustrates many of the important concepts discussed in the Oncoplastic Surgery Overview and speaks directly to the need for breast health care professionals to begin to focus on the role of oncoplastic surgery in both benign and malignant disease. This 12-year-old girl originally presented to her ob/gyn, who then referred her to a general surgeon. The general surgeon did a preoperative workup that included an ultrasound and determined that the mass was exceedingly large and needed to be removed.
The original surgical approach planned by the general surgeon included a periareolar incision extending both medially and laterally across the child's breast. However, rather than take her immediately to surgery, the general surgeon referred this young patient to an oncoplastic surgeon for further evaluation. The oncoplastic surgeon's choice to proceed with an ultrasound-guided core biopsy in order to determine whether this was a benign or malignant lesion was well advised. Although a breast lesion in a 12-year-old child is most likely to be benign, there was a small chance that it could have been a tumor with malignant degeneration or potential, such as a cystosarcoma phyllodes or sarcoma. Thus, performing an ultrasound-guided core biopsy was important prior to surgical planning and intervention.
Once it was determined that the lesion was benign, the surgical plan proceeded in a totally different manner than was recommended by the general surgeon, as noted above. As in the procedure used to remove a subglandular saline or silicone implant, an inframammary approach was utilized. Electrocautery was used to dissect up to, and around the lesion, which was easily identifiable. The lesion was carefully dissected free from the surrounding breast tissue and ultimately was removed completely by inverting the breast. A drain was placed during surgery and the incision was closed without any difficulty. Postoperatively, the patient had an excellent cosmetic result with no significant disfigurement and no loss in the sensation of the nipple.
The Oncoplastic Approach Versus the General Surgery Approach
A general surgeon not experienced in performing explantation or removal of breast implants would most likely not attempt the oncoplastic approach used in this young patient. Importantly, however, in the original surgical approach that was planned, an incision in the periareolar region extending both medially and laterally would leave a significant degree of scarring in this 12-year old girl. In addition, tunneling down through the breast tissue in the inferior periareolar area puts the nerve and arterial supply to the nipple at risk, because it is supplied via the 4th and 5th intercostal nerves, which travel upward through the glandular tissue of the breast and can be injured when dissecting through this area. In addition, it was worrisome that the cosmetic outcome could include significant nipple inversion and/or distortion in the inferior aspect if the general surgical approach was utilized.
Oncoplastic surgery is a new and evolving subspecialty area that blends the principles of surgical oncology with aesthetic and reconstructive surgery. Oncoplastic surgery is a field that clearly illustrates the benefits of an interdisciplinary approach to patients with breast health care issues, and is paramount to ensuring that oncologic principles are not compromised while meeting the needs and desires of the patient from an aesthetic perspective.
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BREAST SURGICAL ONCOLOGY
Daniel Weingrad, MD
The presence of a breast mass is an emotionally devastating and frightening experience for a teenager and her family who must be reassured immediately that this is most likely a benign condition that can be treated in most cases with limited, if any, deformity or long-term consequence. Once developmental deformities such as gigantomastia and asymmetrical hypertrophy have be ruled out, the differential diagnosis of an isolated rapidly enlarging mass in the breast includes juvenile fibroadenoma, phyllodes tumor, rare breast tumors such as rhabdomyosarcoma or mucocele-like tumor, and secondary tumors metastatic to the breast.1,2 The overwhelming majority of cases are juvenile fibroadenoma or phyllodes tumor.3 Rarely atypical hyperplasia and even carcinoma have been reported to occur within fibroadenomas in females younger than 20 years old.4,5 Review of the SEER data reveal fewer than 0.1 cases per 100,000 of breast carcinoma in females less than 20 years of age.6
A prudent preoperative assessment includes breast ultrasound and possibly a core biopsy. MRI of the breast might be useful as it is an important means of diagnosis of soft tissue lesions at other sites. Treatment follows the principles of breast surgery from the perspective of a surgical oncologist. They are: evidence- and outcome-based treatment that maximally conserves breast appearance and function. For a 12-year-old girl a transverse incision through the areola and subjacent developing breast does not respect these principles. Over the last 25 years as a surgical oncologist practicing breast surgery I have shamelessly borrowed plastic surgical techniques to achieve the optimal cosmetic outcome while adhering to sound oncologic practice. This might involve volume displacement techniques for one-stage local reconstruction after partial mastectomy or one-stage chest wall reconstruction with an external oblique myocutaneous flap after extended radical mastectomy for locally advanced tumor. I suppose in this way I have worn the mantle of an "oncoplastic surgeon" somewhat like the fabled emperor wore his new clothes.
There are actually a number of surgical options that have been used to address the problem faced by this patient. For extremely large tumors, excision with reconstruction using the traditional mastopexy approach may be the only practical option.7,8 Retromammary excision through an inframammary incision is a satisfactory approach that meets the goals of oncoplastic surgery.9 For tumors with the appropriate characteristics minimally invasive approaches such as endoscopic extirpation or removal using a plastic bag may be preferable.10,11
After tumor removal the plan for follow-up can be guided by the final histological appearance of the tumor. For juvenile fibroadenoma and benign phyllodes tumors yearly exam by the patient's gynecologist or primary physician is probably sufficient.
References
1. Berardi R, Latini L, Santinelli A et al. Mucocele-like tumor of the breast. Journal of Experimental Clinical Cancer Research 2003, 22, 329-332.
2. Chateil JF, Arboucalot F, Perel Y et al. Breast Metastases in Adolescent girls: US findings. Pediatric Radiology 1998, 28, 832-835.
3. Ciftci AO, Tanyel FC, Buyukpamukcu N et al. Female breast masses during childhood: a 25-year review. European Journal of Pediatric Surgery 1998, 8, 67-70.
4. Diaz NM, Palmer JO, McDivitt RW. Carcinoma Arising in Fibroadenomas of the Breast. American Journal of Clinical Pathology 1991, 95, 614-622.
5. Mies C, Rosen PP. Juvenile Fibroadenoma with Atypical Epithelial Hyperplasia. American Journal of Surgical Pathology 1987, 11, 184-190.
6. Shannon C, Smith IE. Breast Cancer in Adolescents and Young Women. European Journal of Cancer 2003, 39, 2632-2643.
7. McGrath MH. Benign Tumors of the Teenage Breast. Plastic and Reconstructive Surgery 2000, 105, 218-222.
8. Wechselberger G, Schoeller T, Piza-Katzer H. Juvenile fibroadenoma of the breast. Surgery 2002,132, 106-107
9. Simmons RM, Cance WG, Iacicca MV. A Giant Juvenile Fibroadenoma in a 12-Year-Old Girl: A Case for Breast Conservation. The Breast Journal 2000, 6, 418-420.
10. Kitamura K, Hashizume M, Sugimachi K, et al. Early Experience of Endoscopic Extirpation of Benign Breast Tumors via an Extra-Mammary Incision. American Journal of Surgery 1998, 176, 235-238.
11. Rojananin S, Ratanawichitrasin A. Limited incision with plastic bag removal for a large fibroadenoma. British Journal of Surgery 2002, 89, 787-788.
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About the authors
Gail Lebovic, MD, is a breast oncoplastic surgeon and lead surgeon at Baylor Breast Care Specialists of North Texas. Dr. Lebovic serves as the ASBD's secretary- treasurer.
Adeboye O. Osunkoya, MD, is a pathology resident at the University of Oklahoma Health Sciences Center, Oklahoma City.
Patricia Simmons, MD, directs the Mayo Clinic's Pediatric and Adolescent Gynecology program. She is president of the North American Society for Pediatric and Adolescent Gynecology and Professor in the Mayo Medical School.
Ann D. Thor, MD, is Lloyd E. Rader Professor and Chair, Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma. She currently serves on the ASBD's Board of Directors and chairs its Communications Committee.
Daniel Weingrad, MD, is a surgical oncologist specializing in breast at Mount Sinai Comprehensive Cancer Center, Miami Beach, Florida. He currently serves on the ASBD's Communications Committee and on the Expert Working Group in Treatment for the Society's Clearinghouse on Breast Disease.
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