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treatment. Thus far, intracystic papillary carcinoma has been staged and treated
like ductal carcinoma in situ.3 While this management strategy may be appropriate
in some instances, our patient had invasive papillary carcinoma or DCIS. We discussed the challenge of devising a treatment plan at an interdisciplinary tumor board and came to the conclusion that she should be treated for her invasive ductal carcinoma, regardless of cystic origin.
The pathogenesis of intracystic carcinoma
is not well understood. As it stands, it develop in a pre-existing benign cyst or if
a cyst forms subsequent to tumor necrosis and associated changes.4 The malignant potential of complex cystic masses has been described in the literature with malignancy rates of 23 percent5 and 31 percent6 in two series respectively, most commonly DCIS or invasive ductal carcinoma. An extensive literature search was conducted and only one other case of intracystic invasive ductal carcinoma has been described, though nodal status and biomarkers were not reported.7 The hormone receptor status added another aspect of complexity to our patient’s diagnosis and treatment. Intracystic carcinomas are frequently estrogen
receptor and progesterone receptor positive, contributing to a favorable prognosis with
the ability to use hormone therapy.8 Our patient’s tumor was triple negative, which has poor implications for both prognosis and response to treatment.
Her risk of distant metastatic disease at
her tumor biology and stage. Her medical oncologist recommended an adjuvant chemotherapy regimen, which the patient declined given her functional status and concern for toxicity. She also declined radiation treatment and any routine surveillance imaging or staging studies. While the role of adjuvant chemotherapy, hormone therapy, and radiation has been breast cancers, it is less so for intracystic carcinoma. One review found that Intracystic papillary carcinomas with microinvasion or associated DCIS were much more likely to be treated with endocrine or radiation therapy than carcinomas without those high-risk features.9 A recent study of patients with intracystic papillary carcinoma found that the majority of patients had axillary lymph node sampling performed. Patients who underwent breast conserving therapy with adjuvant radiation had improved survival compared
to those who did not receive radiation.10 It is unclear if our patient’s tumor is more similar to high risk invasive ductal carcinoma or lower risk intracystic carcinoma. As in many complex patient scenarios, the burden of
innovative therapy falls on the practitioner who must balance the risk of over-treatment and toxicity with risk of under-treatment and recurrence. Our patient was not willing to risk further deterioration of her functional
CONCLUSION
Invasive ductal carcinoma arising in
a cyst wall is extremely rare, with few previously reported cases in the literature. The pathogenesis and metastatic potential of these tumors is unknown, presenting
a challenge to devising optimal surgical and medical treatment. Literature supports surgical excision with lymph node sampling and adjuvant radiation therapy
if breast conserving therapy is performed. In patients whose tumors display high
risk features, adjuvant chemotherapy and radiation should be offered after informed patient discussion.
CONTRIBUTING AUTHORS
■ SARAH HIMMELSTEIN, MD is a General Surgery Resident at Christiana Care Health System in Newark, Del.
■ EMILY PENMAN, MD is a Breast Surgeon at the Helen F. Graham Cancer Center, Christiana Care Health System in Newark, Del.
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