Author: Rodrigo Arrangoiz MS, MD, FACS, FSSO

👉Malignant phyllodes tumors carry a high risk of local recurrence if not excised to wide negative margins.

👉How wide these margins should be is a matter of debate with data supporting a negative margin of any width to a negative margin as wide as 2 cm.

👉Currently, the National Comprehensive Cancer Network recommends a margin width of 1cm; however, recent studies suggest margin width is not associated with local recurrence or local recurrence free survival.

👉The addition of radiation therapy when margins are closer than 1 cm or other high-risk features are present can also be a consideration but it is controversial.

👉Most data indicate unacceptably high recurrence rates if the tumor is at the inked margin of a malignant phyllodes tumor excision.

👉Mastectomy is not required unless excision cannot achieve tumor-free margins.

👉The most appropriate margin thickness for borderline or benign phyllodes tumors is even less well-defined.

👉Although achieving widely negative margins can reduce local recurrence rates, malignant phyllodes tumors also have metastatic potential regardless of negative margin status. 

👉REFERENCES

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Clinical Presentation

Phyllodes tumors generally present as a palpable mass or, in women being screened, as an abnormal finding on imaging:

• In one third of patients, these tumors grow rapidly and may fill a substantial portion of the breast, causing thinning of the overlying skin and prominent vascularity

Clinical concern for a phyllodes tumor versus a fibroadenoma usually hinges on a history of rapid growth, large tumor size (> 3 cm), and / or older patient age:

• However, with increased adoption of breast screening and breast awareness, smaller and asymptomatic lesions are being detected more often

Imaging Findings

Most phyllodes tumors appear as lobulated masses on mammography and ultrasonography:

Phyllodes tumors may, however, have irregular margins on ultrasonography, a finding seen more frequently in borderline or malignant tumors than in benign tumors

Magnetic resonance imaging (MRI) has been used to evaluate patients with phyllodes tumors, but the clinical benefit of this approach is unclear as these tumors are generally well delineated by physical examination and / or sonography, and correlation of tumor size among mammography, ultrasonography, and MRI is high

• Papillary lesions of the breast:
  • Are common
• They are highly vascular lesions that are intraductal and may transform into malignant variants
• In benign papillary lesions:
  • A vascular stalk may be demonstrated on color Doppler scanning:
    • While multiple feeding vessels may be seen when imaging malignant papillary lesions
• When papillary lesions infarct:
  • The vascular stalk will not be demonstrated
• The ability to reliably distinguish papilloma, in-situ papillary carcinoma, and invasive papillary carcinoma:
  • Is not possible with ultrasound and is quite challenging even on core biopsy:
    • Open surgical biopsy may need to be performed to distinguish malignant from benign papillary lesions

• An “acorn” cyst is lined with papillary apocrine metaplasia:
  • Which can form a mural nodule:
    • The nodule in an acorn cyst is less echogenic than papillomas or papillary carcinomas:
      • Is usually concave, following the contour of the cyst (thus the appearance of a cap on an acorn) instead of convex, and does not have a vascular stalk
        • The mural nodule caused by papillary apocrine metaplasia:
          • Also would not extend into the duct as the papillary lesion shown in the image does
• Tubular adenomas and fibroadenomas:
  • Have a similar sonographic appearance and are frequently round or oval, although tubular adenomas can be fusiform or spindle shaped:
    • Both lesions occur during reproductive years and would not commonly present as a new finding in a postmenopausal patient
• References
  • Jagmohan P, Pool FJ, Putti TC, Wong J. Papillary lesions of the breast: imaging findings and diagnostic challenges. Diagn Interv Radiol. 2013;19(6):471-478.
  • Wyss P, Varga Z, Rössle M, Rageth CJ. Papillary lesions of the breast: outcomes of 156 patients managed without excisional biopsy. Breast J. 2014;20(4):394-401.
  • Stavros AT. Breast Ultrasound. Philadelphia, PA: Lippincott Williams & Wilkins; 2004.

• The rapid growth of this lesion:
  • Suggests that it is a phyllodes tumor:
    • Although a giant fibroadenoma is another possibility
• The most recent ultrasound image shows:
  • An isoechoic, heterogeneous mass that contains cystic, fluid filled spaces, and is vascular on Doppler examination
• In most cases, benign phyllodes tumors:
  • Have margins that are well-circumscribed, and a thin, echogenic capsule is demonstrable
  • The doubling time for a:
    • Benign phyllodes tumor:
      • Is about four months
    • Malignant phyllodes tumor:
      • Is a little over a month
  • Rapidly growing phyllodes tumors:
    • Whether benign or malignant:
      • Often cause prominent veins on the skin:
        • From the developing vascularity
  • Phyllodes tumors are more common in women of Mexican descent:
    • Latin American women with phyllodes tumors tend to be diagnosed at an earlier age than other women
• A malignant phyllodes tumor:
  • Would in all likelihood have a less distinct border than the lesion shown above
• A core needle biopsy:
  • Cannot reliably distinguish phyllodes tumors from fibroadenomas:
    • It is therefore not sufficient for a definitive diagnosis
• The correct management is excision:
  • With or without a pre-operative core biopsy:
    • With care to completely excise the tumor
• Phyllodes tumors can also be difficult to distinguish from giant juvenile fibroadenomas:
  • But they should also be treated by surgical excision
• The rapid growth of this lesion suggests a phyllodes tumor, not a fibroadenoma
• References:
  • Guillot E, Couturaud B, Reyal F, Curnier A, Ravinet J, Lae M, et al. Management of phyllodes breast tumors. Breast J. 2011;17(2):129-137.
  • Plaza MJ, Swintelski C, Yaziji H, Torres-Salichs M, Esserman LE. Phyllodes tumor: review of key imaging characteristics. Breast Dis. 2015;35(2):79-86.
  • Rajan PB, Cranor ML, Rosen PP. Cystosarcoma phyllodes in adolescent girls and young women: a study of 45 patients. Am J Surg Pathol. 1998;22(1):64-69.
  • Sosin M, Pulcrano M, Feldman ED, Patel KM, Nahabedian MY, Weissler JM, et al. Giant juvenile fibroadenoma: a systematic review with diagnostic and treatment recommendations. Gland Surg. 2015;4(4):312-321.
  • Stavros AT. Atypical, high-risk, premalignant, and locally aggressive lesions. In: Stavros AT. Breast Ultrasound. Philadelphia, PA: Lippincott Williams & Wilkins; 2004:689-711.

• The sonographic image for this patient shows a fairly round, well-circumscribed, microlobulated lesion with posterior enhancement
• The most reliable sonographic feature of medullary carcinoma is:
  • Enhanced through transmission:
    • This results from the highly cellular lesion with a paucity of desmoplastic fibrous tissue, in addition to areas of cystic necrosis and hemorrhage
  • Medullary carcinomas:
    • Are classically well-circumscribed:
      • Though most of them have some area of angularity
  • High-grade, colloid (mucinous), and papillary carcinomas:
    • Typically have a sonographic appearance similar to medullary carcinoma:
• The lesion in Image:
  • Is irregular, has spiculated margins, and shows posterior shadowing:
    • All of these features are commonly seen in fat necrosis, scar, and low-grade invasive cancers

• Hematoma is unlikely to have enhanced through transmission
• References:
  • Harvey JA. Unusual breast cancers: useful clues to expanding the differential diagnosis. Radiology. 2007;242(3):683-694.
  • Meyer JE, Amin E, Lindfors KK, Lipman JC, Stomper PC, Genest D. Medullary carcinoma of the breast: mammographic and US appearance. Radiology. 1989;170(1 Pt 1):79-82.
  • Stavros AT. Malignant solid breast nodules: specific types. In: Stavros AT. Breast Ultrasound. Philadelphia, PA: Lippincott Williams & Wilkins; 2004:297-688.

• Phyllodes tumors:Are true fibroepithelial neoplasms:With both epithelial and stromal components
  • • They represent less than 1% of all breast tumors:Most commonly occurring in women in their 50sThey are rapidly growing tumors:But are usually well-defined / circumscribed:Often appear as distinct masses:Indistinguishable from fibroadenomas on mammography and ultrasound

• • The World Health Organization has divided phyllodes tumors into three categories: BenignBorderline
    • Malignant

• • The majority of phyllodes tumors are benign:With local recurrence rates of 15% to 20% depending on adequacy of excision
  • Grading is usually based on multiple criteria in the stromal component:Nuclear pleomorphismMitotic rate
    • Stromal overgrowth
    • Cellularity
    • Tumor margins
  • The distinction between cellular fibroadenomas and phyllodes tumors:Can be difficult
  • The definition of “optimal” margin varies for various types of phyllodes tumors:Historically, margins of 2 cm to 3 cm have been recommended:But current recommendations are considerably less:With the only “absolute” being that the margin is tumor-free
  • Malignant transformation:Is a rare occurrence
  • Metastases can occur:In a small number of malignant phyllodes tumors and an even smaller number of borderline phyllodes tumors
  • Radiotherapy may be of benefit for local control of malignant phyllodes tumors
  • Differences in grading of phyllodes tumors among pathologists can be substantial

REFERENCES

• Phyllodes tumors of the breast:
  • Are rare lesions
• Phyllodes tumors are classified into three groups:
  • Benign
  • Borderline
  • Malignant
• Borderline and malignant phyllodes tumors:
  • Have a higher incidence of local recurrence:
    • Therefore, historically management was:
      • A mastectomy without nodal assessment
    • Recently, more patients are undergoing lumpectomy for this lesion
    • Researchers believe that local recurrence rates:
      • Are similar for borderline and malignant phyllodes tumors:
• • Historically:
    • 1 cm margins have been recommended for malignant and borderline phyllodes tumors:
      • And continue to be recommended in the current 2023 NCCN guidelines
    • The guidelines however note that while narrow margins are associated with an increased risk for local recurrence:
      • They are not an absolute indication for mastectomy
  • Newer data may suggest a smaller margin is adequate:
    • Spanheimer et al^:
      • Identified local recurrences in 16% of 71 patients with borderline or malignant phyllodes tumors undergoing:
        • Breast-conserving surgery
      • Some of these patients had a positive or close (< 1 mm) margin
      • When the subset of patients with a margin > 1 mm was considered:
        • The local recurrence rate was 12%
  • In addition, radiation may play a role in decreasing the risk of local recurrence:
    • In a prospective, multi-institutional study, 46 patients (30 with malignant phyllodes tumors and 16 with borderline phyllodes tumors) underwent margin-negative resections followed by radiation therapy:
      • Eight of these patients had margins < 2 mm
      • After 10 years of observation for all patients:
        • None had developed a local recurrence
    • In another study using data from the National Cancer Database, Gnerlich et al:
      • Showed that adjuvant radiation therapy decreased the risk of local recurrence after resection of phyllodes tumors by:
        • More than half (hazard ratio [HR] 0.43, 95% confidence interval [CI] 0.19–0.95).⁴
• References
  • Tan, BY, Acs, G, Apple, SK, et al. Phyllodes tumor of the breast: a consensus review. Histopathology. 2016;68(1):5-21.
  • Spanheimer P, Murray M, Zabor E, et al. Long term outcomes after surgical treatment of malignant/borderline phyllodes tumors of the breast. Ann Surg Oncol. 2019;26(7):2136-2143.
  • Barth R, Wells W, Mitchell S, Cole B. A prospective, multi-institutional study of adjuvant radiation therapy after resection of malignant phyllodes tumors. Ann Surg Oncol. 2009;16(8):2288-2294.
  • Gnerlich J, Williams R, Yao K, Jaskowiak N, Kulkarni S. Utilization of radiotherapy for malignant phyllodes tumors: analysis of the National Cancer Database 1998–2009. Ann Surg Oncol. 2014;21(4):1222-1230

#Arrangoiz #BreastSurgeon #CancerSurgeon #Teacher #SurgicalOncologist #BreastCancer #PhyllodesTumors #Miami #MountSinaiMedicalCenter #MSMC #Miami #Mexico #Doctor #Surgeon

• The sonographic appearance of a normal lymph node:
  • Is elliptical with a thin, hypoechoic cortex and an isoechoic to hyperechoic fatty hilum (Image).

• Metastatic carcinoma in a lymph node:
  • Would usually have an asymmetric thick cortex or have near-total or total obliteration of the hilum:
    • Resulting in a rounded, hypoechoic mass (Image)

• Axillary adenopathy can occur in association with rheumatoid arthritis:
  • But the sonographic findings would usually be a symmetrical, mild thickening of the cortex:
    • Usually with preservation of the hilum
• Silicone granulomas:
  • Classically create a snowstorm appearance:
    • Which allows a definitive diagnosis by ultrasound alone

• The mass in the image has a rounded anterior border and “dirty” incoherent shadowing that obscures the posterior border of the lesion:
  • Nothing other than silicone can cause this sonographic appearance:
    • But it is difficult to distinguish free silicone that has migrated to the axilla from a node that has been replaced with silicone
  • In different stages of development, silicone extravasation can also mimic complex cysts or fibrotic nodules:
    • Depending on the amount of silicone extravasated and the amount of time from extravasation
  • If a large amount of silicone is released into the tissue:
    • The ultrasound pattern is that of a complex cyst
  • Silicone’s presence in tissues can cause a local inflammatory reaction:
    • Which may cause tissue fibrosis and a fibrotic nodule:
      • This is a late finding seen following extravasation
• References
  • Berg WA, Caskey CI, Hamper UM, Anderson ND, Chang BW, Sheth S, et al. Diagnosing breast implant rupture with MR imaging, US, and mammography. Radiographics. 1993;13(6):1323-1336.
  • Juanpere S, Perez E, Huc O, Motos N, Pont J, Pedraza S. Imaging of breast implants‒a pictorial review. Insights Imaging. 2011;2(6):653-670.

• The sonographic appearance of a normal lymph node:
  • Is elliptical with a thin, hypoechoic cortex and an isoechoic to hyperechoic fatty hilum (Image).

• Metastatic carcinoma in a lymph node:
  • Would usually have an asymmetric thick cortex or have near-total or total obliteration of the hilum:
    • Resulting in a rounded, hypoechoic mass (Image)

• Axillary adenopathy can occur in association with rheumatoid arthritis:
  • But the sonographic findings would usually be a symmetrical, mild thickening of the cortex:
    • Usually with preservation of the hilum
• Silicone granulomas:
  • Classically create a snowstorm appearance:
    • Which allows a definitive diagnosis by ultrasound alone

• The mass in the image has a rounded anterior border and “dirty” incoherent shadowing that obscures the posterior border of the lesion:
  • Nothing other than silicone can cause this sonographic appearance:
    • But it is difficult to distinguish free silicone that has migrated to the axilla from a node that has been replaced with silicone
  • In different stages of development, silicone extravasation can also mimic complex cysts or fibrotic nodules:
    • Depending on the amount of silicone extravasated and the amount of time from extravasation
  • If a large amount of silicone is released into the tissue:
    • The ultrasound pattern is that of a complex cyst
  • Silicone’s presence in tissues can cause a local inflammatory reaction:
    • Which may cause tissue fibrosis and a fibrotic nodule:
      • This is a late finding seen following extravasation
• References
  • Berg WA, Caskey CI, Hamper UM, Anderson ND, Chang BW, Sheth S, et al. Diagnosing breast implant rupture with MR imaging, US, and mammography. Radiographics. 1993;13(6):1323-1336.
  • Juanpere S, Perez E, Huc O, Motos N, Pont J, Pedraza S. Imaging of breast implants‒a pictorial review. Insights Imaging. 2011;2(6):653-670.

• The mammogram shows a spiculated mass with extensive edema:
  • As evidenced by skin thickening and increased trabecular markings in the breast (Images)
• Ultrasound shows a heterogeneous, irregular, hypoechoic mass with some angular and spiculated margins (Image):
  • Internal vascularity is demonstrated on Doppler examination (Image)
• Gynecomastia:
  • Does not cause erythema, skin thickening, and breast edema:
    • The mammogram would usually show a triangular shaped density radiating from the nipple into the breast tissue (Image)

• The sides of the mammographic triangle in gynecomastia are:
  • Usually straight or concave (Image) as opposed to convex in breast cancer
• The sonographic appearance of gynecomastia depends on the phase of development:
  • It can appear quite hypoechoic and have angular margin
• Paget’s disease of the breast is rare, but it does occur in males:
  • It would present with a nipple rash and would not be expected to have secondary infection
  • There are usually no mammographic or sonographic features unless there is an underlying invasive carcinoma
• References
  • Abeywardhana DY, Nascimento VC, Dissanayake D, Taylor D, Metcalf C, Saunders C, et al. Review of ultrasound appearance in inflammatory breast cancer: a pictorial essay. J Med Imaging Radiat Oncol. 2016;60(1):83-87.
  • Stavros AT. Evaluation of the male breast. In: Stavros AT. Breast Ultrasound. Philadelphia, PA: Lippincott Williams & Wilkins; 2004:712-741.
  • Khalkhali I, Cho J. Male breast cancer imaging. Breast J. 2015;21(3):217-218.
  • Ottini L, Palli D, Rizzo S, Ferico M, Bazan V, Russo A. Male breast cancer. Crit Rev Oncol Hematol. 2010;73(2):141-155.