8) Intracystic papillary neoplasm – A rare breast tumor!

  [6] RAD-TIMES -- VOLUME 1-- ISSUE 6

6) Intracystic papillary neoplasm – A rare breast tumor!

-By Dr. Suchita Durge (Senior Resident) & Dr. Sneha Deshpande (Assistant Professor)

Clinical profile– A 45 year old lady presented with a painless palpable lump in her right breast since 2 years. There was no abnormal nipple discharge or nipple retraction. No significant past medical/ surgical history or family history of breast cancer. Physical examination revealed a firm, well-circumscribed mass in the upper inner quadrant of right breast measuring approximately 1x 1 cm. The mass was mobile and it was not associated with nipple retraction or overlying skin changes. The left breast and both axillae were normal. She was referred for a diagnostic mammography.

Imaging: Mammography (a-d) with Tomosynthesis (e): revealed a well circumscribed round high density mass in the lower inner quadrant of right breast which measured approximately 1 x 1 x1 cm, approximately 5 cm from the nipple. It showed a focal contoural bulge along its cranial aspect with few spiculations, which was better appreciated on tomosynthesis. The lesion was not associated with abnormal microcalcifications.

USG (f) with Color Doppler imaging (g) and US elastography (h): revealed a well circumscribed complex cystic mass lesion with posterior acoustic enhancement at 3 o’clock position, 5 cm from nipple. It showed heterogenous hypoechoic papillary solid component within measuring approximately 8×7 mm, with mild internal vascularity. This solid component was seen focally extending beyond the cyst margin. US elastography showed a Strain ratio of 3.24.

Diagnosis– Intracystic papillary neoplasm of right breast. Excision biopsy was done which showed intracystic papillary carcinoma.

Discussion:Intra-cystic Papillary Neoplasm (ICPN) is a rare breast tumor seen commonly in post-menopausal women, which accounts for approximately 1%–2% of all breast cancers. (1) It shows less aggressive clinical behavior than invasive ductal carcinoma, with a lower incidence of lymph node metastases. ICPN can present as an in situ or invasive lesion and may be associated with ductal carcinoma in situ and/or invasive ductal carcinoma. (2,3) Clinically, these patients may present with painless palpable mass &/or bloody nipple discharge or with an incidentally detected radiological abnormality. Retraction of the nipple and skin may be an associated clinical finding, in case of large tumors.

On mammography-ICPN commonly appears as a round to oval hyperdense lesion, with well-circumscribed margins. Some lesions may present as irregular masses with obscured, indistinct or spiculated margins, if associated with perilesional inflammation or invasion. This tumor may be uni- or multifocal and may occasionally be associated with microcalcifications. (2,4-8) ICPCs tend to be larger than intracystic papillomas. On USG, ICPNs commonly appear as single or multiple predominantly cystic masses with solid papillary areas projecting into the cyst lumen with or without septae. Color Doppler sonography shows blood flow in the solid component. The presence of fluid-debris level in the cyst is usually related to spontaneous bleeding. Size of the intracystic solid component along with its irregular borders & heterogeneous echogenicity are good predictors of its malignant potential. (2,4-8)

Intracystic papillomas or ICPCs may be diagnosed with sonography if they do not occupy the entire cyst lumen, or else, they are not distinguishable from other solid masses. Speer et al. (9) evaluated 40 patients diagnosed with intracystic papillary carcinoma and found that the predominant mammography features were oval shape (57%), obscured margins (40%), and high density (67%). The predominant ultrasound features were oval masses followed by irregular masses. 35% patients presented with solid cystic masses. They found that there were no specific imaging features to differentiate in situ vs invasive IPC.

Definitive tissue diagnosis is a must. The mainstay of treatment is surgical resection, with adjuvant therapy if associated with DCIS or invasive carcinoma.

References:

1. Rosen PP. Papillary carcinoma. Rosen’s Breast Pathology. 1197:335-54.
2. Rodríguez MC, Secades AL, Angulo JM. Best cases from the AFIP: intracystic papillary carcinoma of the breast. Radiographics. 2010 Nov;30(7):2021-7.
3. W.W.M. Lam, A.P.Y.Tang, G. Tse and W.C.W.Chu, “Radiology-pathology conference: papillary carcinoma of breast,” Clinical Imaging  Vol. 29 no.6 pp. 396-400. 2005.
4. M.J. Brookes and A. G. Bourke, “Radiological appearance of papillary breast lesions”, Clinical Radiology, Vol. 63 no.11, pp. 1265-1273, 2008.
5. K Mugler, C Marshal, L Hardesty, C Finlayson, M Singh. Intracystic papillary carcinoma of the breast: differential diagnosis and management Oncology, 21 (7) (2007), pp. 871-876
6. KI Bland, EM Copeland The breast: comprehensive management of benign and malignant disorders (3rd ed.), Saunders, Philadelphia (2004), p. 636
7. B Dogan, G Whitman, Lavinia Middleton, M Phelps. Intracystic papillary carcinoma of the breast Amer J of Roen, 181 (1) (2003), p. 186
8. D Kopans Breast imaging (3rd ed.), Lippincott Williams & Wilkins, Philadelphia (2007), pp. 860-861
9. Speer ME, Adrada BE, Arribas EM, Hess KR, Middleton LP, Whitman GJ. Imaging of Intracystic Papillary Carcinoma. Curr Probl Diagn Radiol. 2019 Jul -Aug;48(4):348-352.

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