| Literature DB >> 27363851 |
Kimberly A Chun1, Philip R Cohen2.
Abstract
BACKGROUND: Basal cell carcinomas (BCCs) usually occur in sun-exposed areas. However, they may also occur-albeit infrequently-in unusual locations, such as the nipple-areola complex.Entities:
Keywords: Areola; Basal cell carcinoma; Nipple; Review
Year: 2016 PMID: 27363851 PMCID: PMC4972734 DOI: 10.1007/s13555-016-0128-3
Source DB: PubMed Journal: Dermatol Ther (Heidelb)
Fig. 1a, b A previously unreported clinical presentation of basal cell carcinoma of the areola. Distant view (a) of a flesh-colored to hypopigmented dermal nodule on the upper medial quadrant of the right areola in a 67-year-old Caucasian male with Fitzpatrick skin type 2. He had a prior history of basal cell carcinoma on the left arm diagnosed 3 years earlier and presented with a 6-month history of a slowly enlarging, asymptomatic lesion on the right areola and adjacent breast. He had a prior history of moderate sun exposure as a young adult. He had no exposure to ionizing radiation and had no family history of basal cell carcinoma or basal cell nevus syndrome. Closer view (b) of the right breast shows the 7 × 7-mm flesh-colored to hypopigmented dermal nodule on the upper medial quadrant of his right areola and extending into the adjacent breast. A 3-mm punch biopsy was performed. The patient in these figures is also cited in Table 1 (case 35)
Fig. 2a, b A previously unreported pathologic presentation of the basal cell carcinoma of the areola shown in Fig. 1. Low (a) and higher (b) magnification views of the pigmented basal cell carcinoma show nodular aggregates of basaloid tumor cells extending from the epidermis into the dermis (a). Deposits of melanin were present not only in the tumor cells but also in dermal melanophages (b). The residual tumor was excised using the Mohs micrographic technique, and clear margins were achieved after three stages. The final wound measured 20 × 14 mm and a layered side-to-side closure was used to close the surgical defect. The patient in these figures is also cited in Table 1 (case 35)
Clinical characteristics of males with basal cell carcinoma of the nipple-areola complex
| Case | Age (years) | Race | Loc | Morphology | Hist sub | P | Other NMSCs | Risk factors | Rx | Outcome | References |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 60 | NS | R N, A | Irregular ulcerated patch | NS | – | NS | NS | Simple excision and axillary LyN sampling | No recurrence Died 18 months later of unrelated cause | Robinson [ |
| 2 | 57 | NS | L N | NS | NS | NS | NS | NS | NS | LyN involvement Died of disease in 8 years | Wainwright [ |
| 3 | 43 | W | N | NS | Nod | +a | NS | NS | Simple mastectomy | No recurrence after 1 year | Congdon et al. [ |
| 4 | 49 | NS | R N | Scaly and crusted lesion with ulceration and scarb | NS | NS | NS | NS | Wide excision | NS | Farrow [ |
| 5 | 71 | W | R N, A | 8-cm tumor with ulceration | Nod | – | NS | NS | Simple mastectomy | LyN involved After mastectomy, no recurrence | Wyatt [ |
| 6 | 72 | NS | R N | Enlarged, firm R nippleb | Nod | – | NS | NS | Wide excision | NS | Lupton et al. [ |
| 7 | 72 | NS | L N, A | NS | NS | NS | NS | NS | NS | NS | C2, Rahbari et al. [ |
| 8 | 47 | NS | L A | NS | NS | NS | NS | NS | NS | NS | C22, Rahbari et al. [ |
| 9 | 49 | W | R A | Pink, red plaque | NS | NS | NS | NS | MMS | Clear margins at surgery | Robins et al. [ |
| 10 | 53 | W | L N | Ulcerated lesionb | Nod | – | NS | NS | Simple excision | Clear margins at surgery | Knudsen [ |
| 11 | 51 | W | L N, A | Reddish brown, scaly, indurated plaque with pearly rolled border | S | – | None | Sun exposure | Simple excision | Clear margins at surgery | Bruce et al. [ |
| 12 | 58 | NS | L N | Skin breakdown of L nippleb | M: Nod, I | – | None | None | Simple excision followed by radiotherapy | 4 Years later had recurrence in axillary LyN, treated with mastectomy and LyN clearance | Shertz et al. [ |
| 13 | 86 | NS | R N | Red, scaly, ulcerated oozing lesionb | F | – | BCC and SCC on face and scalp | Trauma to the chest | Simple excisiond | NS | Nirodi et al. [ |
| 14 | 63 | W | R N, A | Eroded plaque with crust | Nod | – | NS | None | Simple excision | No recurrence after 16 months | C1, Cain et al. [ |
| 15 | 80 | W | R N, A | Indurated reddish brown mass with ulcerationb | F | – | NS | NS | Wide excision | No recurrence after 8 months | C2, Cain et al. [ |
| 16 | 68 | W | L N | 2 cm reddish brown to gray and blackish bluish nodule | Sup | +e | NS | NS | Simple excision | Clear margins at surgery | Titzmann et al. [ |
| 17 | 57 | W | R N | 1.5 cm papule | Nod | – | BCC × 4 on face and arms | Sun exposure, although noted patient states he always wore a shirt | Simple mastectomy | No recurrence after 2 years | Benharroch et al. [ |
| 18 | 55 | NS | N, A | NS | BCC | NS | NS | NS | MMS | NS | Weber et al. [ |
| 19 | 57 | W | R N | Flesh colored, indurated papule | Nod | – | None | None | Wide excision | No recurrence after 5 years | C1, Betti et al. [ |
| 20 | 39 | W | N, A | Well-limited plaque with crust | Sup | – | NS | None | Wide excision | No recurrence after 2 years | C2, Betti et al. [ |
| 21 | 67 | W | L N,A | Ulcerated, erythematous lesionb | Nod | – | BCC forehead | NS | Wide excision | Clear margins at surgery | Gupta et al. [ |
| 22 | 61 | W | L A | Pink, pearly papule | NS | NS | BCC × 2 on abdomen | Burn to chest wall from fire | MMS | Clear margins at surgery | Nouri et al. [ |
| 23 | 78 | NS | L N, A | Erythematous nodule with crusting and swelling | Nod | – | NS | None | Simple mastectomy with LyN dissection | Clear at surgery | Kim et al. [ |
| 24 | 60 | W | L N | Erythematous ulcer with scale and crustb | F | – | None | None | Simple mastectomy | Clear at surgery | Avci et al. [ |
| 28 | 69 | W | L N, A | Erythematous plaque with crust | NS | NS | NS | NS | MMS | No recurrence after 6 months | Cummins et al. [ |
| 25 | 60 | W | L N | Enlarged L nippleb | M: S, MN | – | BCCs | Sun | Refused excision | Lost to follow-up | Oram et al. [ |
| 26 | 76 | W | L N | Ulcerated lesion on L nippleb | Nod | – | NS | NS | NS | NS | Kacerovska et al. [ |
| 27 | 69 | W | R N | Poorly defined, pink, telangiectatic plaque with ulceration | M: Nod, S | – | None | Sun exposure | MMS | No recurrence after 2 years | Sinha et al. [ |
| 29 | 42 | W | L N, A | Poorly defined erythematous plaque | M: Nod, S | – | BCCs | BCNS, immune-suppressed | MMS | No recurrence after 3 months | Williams et al. [ |
| 30 | 23 | NS | R N | Well-defined pink, semicircular papule with multiple pin-point pigmented macules on the nipple | NS | +e | NS | NS | Simple excision | No recurrence after 5 years | Brown et al. [ |
| 31 | 78 | Asian | L N, A | Pigmented macule | Nod | +e | None | None | Simple excision | Disease free at 1 year | Kalyani et al. [ |
| 32 | 78 | Asian | R N | Pigmented mass with ulcerationb | Sb | +e | NS | Gastric cancer, chemo | Simple excision | No recurrence at 6 months | Takeno et al. [ |
| 33 | 75 | W | R N | Erythematous ulcer on R nippleb | S | – | Surgical excision of nod BCC 3 years prior | NS | MMS showed lactiferous ducts were infiltrated, so simple mastectomy was performed | Clear margins after simple mastectomy | Miglino et al. [ |
| 34 | 65 | NS | R N | Irregular, black macule | S | +e | NS | NS | NS | NS | Kitamura et al. [ |
| 35 | 67 | W | R A | Flesh colored to Hypopigmented nodule | Nod | +h | BCC of L arm | None | MMS | No recurrence after 6 months | Chun et al.i CR |
A areola, BCNS basal cell nevus syndrome, Cm centimeter, CR current report, F fibroepithelioma of pinkus, Hist sub histologic subtype, I infiltrative, L left, Loc location, LyN lymph node, M mixed, MMS Mohs micrographic surgery, MN micronodular, N nipple, Nod nodular, NS not stated, P pigmented; S superficial, W white, Y years
aTumor is microscopically pigmented. Clinical presentation was not stated
bPrimary morphology not stated
cArticle in Danish
dFor this patient, the nipple was excised
eTumor is both clinically and microscopically pigmented
fArticle in German
gArticle in Korean
hTumor is microscopically pigmented, but not clinically pigmented
iPresented in Figs. 1 and 2
jSimple excision is defined as excision with narrower margins if visually normal-appearing skin
kWide local excision is defined as involving a larger margin of normal-appearing skin
Clinical characteristics of females with basal cell carcinoma of the nipple-areola complex
| Case | Age (years) | Race | Loc | Morphology | Histo | P | Other NMSCs | Risk factors | Rx | Outcome | References |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NS | NS | N | NS | NS | NS | NS | NS | Simple mastectomy | Alive and disease free at 2 years | Congdon et al. [ |
| 2 | 66 | W | L N | Red, scaly nipple with indurationa | S | – | NS | NS | Wide excision | Clear margins at surgery | Davis et al. [ |
| 3 | 67 | W | L N, A | Erythematous, eczematous nodule | S | – | None | Smoker | Etretinate followed by simple mastectomy | Clear margins at surgery | Jones et al. [ |
| 4 | 49 | NS | N, A | NS | NS | NS | NS | NS | No treatment | NS | Betti et al. [ |
| 5 | 71 | W | Bilateral A | Scaly plaques. Plaque on left breast with ulceration | S | NS | BCC, melanoma | NS | Wide excision | No recurrence after 6 months | Wong et al. [ |
| 6 | 35 | NS | L A | Red plaque with well-defined borders | S | – | None | None | Simple excision | No recurrence after 1 year | Nunez et al. [ |
| 7 | 75 | NS | L N, A | Red, eczematous lesiona | M: Nod, S | – | NS | NS | Wide excision followed by radiotherapy | Clear margins at surgery | Sauven et al. [ |
| 8 | 65 | NS | L N, A | Erythematous, indurated plaque with ulceration | M: Nod, I | – | None | Moderate sun exposure | MMS | No recurrence after 1 year | Sanchez- Carpintero et al. [ |
| 9 | 82 | Asian | L A | Dark brown papule | Nod | +b | None | None | Wide Excision | Disease free after 2 years | Yamamoto et al. [ |
| 10 | 47 | H | L N, A | Ill-defined erythematous plaque | S | – | None | None | MMS | Clear margins at surgery | Zhu et al. [ |
| 11 | 47 | W | L N, A | Red-brown, indurated nodule | Nod | – | None | None | Wide excision | No recurrence after 2 years | C3, Betti et al. [ |
| 12 | 46 | Asian | R A | Well-defined, slightly crusted plaque | S | – | None | None | Initially had simple excision, but due to positive margins had a simple mastectomy and excision of an axillary sentinel LyN | Disease free after 1 year | Huang et al. [ |
| 13 | 49 | W | L N,A | Indurated red-brown plaque | M: S, I | – | None | Topless sunbathing | MMS with sentinel LyN biopsy | Clear margins at surgery | Rosen et al. [ |
| 14 | 74 | NS | R N | Eczematous lesiona | S | – | NS | NS | Simple excision | Clear margins at surgery | Chu et al. [ |
| 15 | 67 | Asian | R A | Black hyperkeratotic plaque | Nod | +b | None | None | Wide excision | Disease free after 14 months | Jung et al. [ |
| 16 | 48 | NS | R N, A | Hyperpigmented, erythematous plaque with ulceration | Nod | +b | None | None | Simple excision | Clear margins at surgery | Sharma et al. [ |
| 17 | 72 | Asian | R N, A | Erythematous ulcer with scale and crusta | F | – | NS | NS | Wide excision | NS | Xu et al. [ |
| 18 | 40 | W | R N | Ulcerated nodule | Nod | – | None | None | Simple excision | No recurrence after 18 months | Trignano et al. [ |
| 19 | 66 | AA | L A | Lichenified, scaly, excoriated plaque | S | +e | NS | NS | 5-FU BID x 6 weeks | Resolved clinically, but recurred after 22 months | Goddard et al. [ |
| 20 | 82 | W | L N | Nodule | Nod | – | NS | NS | Wide excision | No recurrence after 3 years | Ozerdem et al. [ |
A areola, AA African American, F fibroepithelioma of pinkus, Hist sub histologic subtype, I infiltrative, L left, Loc location, LyN lymph node, M mixed, MMS Mohs micrographic surgery, N nipple, Nod nodular, NS not stated, R right, S superficial, Y years
aPrimary morphology not stated
bTumor is both clinically and microscopically pigmented
cSimple excision is defined as excision with narrower margins if visually normal-appearing skin
dWide local excision is defined as involving a larger margin of normal-appearing skin
eTumor is microscopically pigmented, but not clinically pigmented
Initial treatment of basal cell carcinomas of the nipple-areola complex
| Initial treatmenta | Malesb | Femalesnc | Totald |
|---|---|---|---|
| Simple excision | 10e | 5f | 15 |
| Wide excision | 6 | 8g | 14 |
| MMS | 8h | 3 | 11 |
| Simple mastectomy | 5 | 1i | 6j |
| 5-Fluorouracil | 0 | 1k | 1 |
| Etretinate | 0 | 1l | 1 |
| No treatment | 1 | 1 | 2 |
| Total | 30 | 20 | 50 |
MMS Mohs micrographic surgery
aInitial treatment not stated in 5 males
bNumber of males for whom treatment was performed
cNumber of females for whom treatment was performed
dTotal number of males and females for whom treatment was performed
eThis group includes one patient whose nipple was excised, and the excision type was not specified. Two patients had subsequent treatment including either axillary lymph node sampling or radiotherapy
fOne female had additional treatment: simple mastectomy and lymph node sampling
gOne female had additional treatment: radiotherapy
hFollowing MMS, one male had a simple mastectomy
iOne female was treated with etretinate and subsequently had a simple mastectomy
jSimple mastectomy was the initial treatment for six patients; however, nine mastectomies were eventually performed. One male was initially treated with MMS, and one female initially treated by simple excision followed by radiotherapy; one female was initially treated with etretinate
kOne female was treated with 5-fluorouracil twice daily for 6 weeks. The BCC initially resolved, but recurred at 22 months
lFollowing etretinate therapy, one female had a simple mastectomy