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. 2020 Feb 14;24:19.084. doi: 10.7812/TPP/19.084

Primary Breast Carcinoma of the Vulva Metastatic to Lymph Nodes and Bones: A Case Report and Literature Review

Aneesha Ananthula 1,✉, Blake Lockwood 2, John Savage 3, Sharp Malak 4, Chien Chen 5, Issam Makhoul 2, Angela Pennisi 2
PMCID: PMC7039421  PMID: 32097114

Abstract

Introduction

Primary breast carcinoma can occur at ectopic sites. The axilla is the most common site of ectopic primary breast cancer, but presentation in the vulva is rare. We discuss a rare presentation of primary breast carcinoma of the vulva with distant lymph node and bone metastases in a premenopausal woman.

Case Presentation

A vulvar malignancy consistent with adenocarcinoma of the mammary gland type was diagnosed in a 47-year-old premenopausal woman. The patient underwent radical vulvectomy with bilateral superficial and deep inguinal lymphadenectomy. The tumor was positive for estrogen receptor and negative for progesterone receptor and human epidermal growth factor receptor 2/neu on immunohistochemical findings. A positron emission tomography-computed tomography scan demonstrated lymph node and bone metastases. Her disease was treated as stage IV breast cancer with metastases to the bone. Palliative treatment with ovarian suppression, aromatase inhibitor, and cyclin-dependent kinase 4/6 inhibitor was recommended.

Discussion

For a diagnosis of primary breast cancer of the vulva, a thorough metastatic workup should be performed, with attention directed toward detecting a breast primary disease by results of the history, physical examination, and radiologic examination of the breasts mainly to help confirm that the vulvar lesion is the primary site as opposed to metastasis from a breast primary cancer and also for staging. Management of this rare entity is challenging because of a lack of specific guidelines, and treatment, therefore, is similar to that of breast cancer.

Treatment should consist of an individualized combination of surgery, radiotherapy, chemotherapy, and antiestrogen hormonal therapy.

Keywords: adenocarcinoma of mammary-like glands in the vulva, ectopic primary breast cancer, primary breast carcinoma of the vulva

INTRODUCTION

Ectopic breast tissue can occur anywhere along the primitive milk line, extending from the axilla to the groin, as a result of incomplete involution of the ectodermal mammary ridges during embryologic development.1–3 Primary breast carcinoma can occur at ectopic sites. The axilla is the most common site of ectopic primary breast cancer, but presentation in the vulva is rare. To date, approximately 36 cases of ectopic primary breast carcinomas of the vulva have been reported in the literature. Here, we discuss a rare presentation of primary breast carcinoma of the vulva with distant metastases to lymph nodes and bone in a premenopausal woman.

CASE PRESENTATION

Presenting Concerns

A 47-year-old, multiparous, premenopausal woman was referred to the Gynecologic Oncology Department at our institution with a diagnosis of a vulvar malignancy. The patient noted a mass in the right vulva approximately 1 year before presentation. She presented to her local gynecologist about 6 months later when she noted more swelling, pain, and some bleeding from the mass. Excision of the right labium majus was performed. The patient was otherwise healthy and had no remarkable medical history.

A review of pathologic slides from her presenting institution showed a 2.7-cm adenocarcinoma invading subcutaneous tissue, epidermis, and dermis with skin ulceration. Multifocal lymphovascular and extensive perineural invasion was noted. Deep surgical margins were involved by adenocarcinoma, but the peripheral surgical margins were clear. Immunohistochemical stains demonstrated that the adenocarcinoma was diffusely positive for GATA-3 and negative for CK7, CK20, CDX2, PAX-8, and CD56. The morphologic characteristics and immunophenotype were consistent with adenocarcinoma of mammary gland type vs a poorly differentiated vulvar adnexal tumor (less likely) and was considered to either originate from the vulva or be metastatic from the breast. No benign ectopic breast tissue was identified.

Therapeutic Intervention and Treatment

The patient underwent radical vulvectomy with bilateral superficial and deep inguinal lymphadenectomy. Final pathologic findings showed multiple (4/17) matted lymph nodes positive for carcinoma with extracapsular extension and residual grade 3, mammary-type adenocarcinoma. Focal ectopic breast tissue, separate from the residual tumor, was identified. Surgical margins were negative for cancer. Estrogen receptor expression was strong, with diffuse nuclear positivity. Progesterone receptor and human epidermal growth factor receptor 2 (HER2/neu) were negative on immunohistochemical testing. The patient was referred to a medical oncologist for further management.

A positron emission tomography-computed tomography (PET/CT) scan was performed to complete the staging. The scan showed multiple right iliac lymph nodes with mild to moderate activity and a 1-cm lytic lesion at the first thoracic vertebral body with moderate activity (standardized uptake value maximum of 2.2), which was concerning for metastatic disease (Figures 1 and 2). A biopsy of pelvic nodes or bone was requested, but was not feasible. Results of imaging of the brain were negative for metastases. A bilateral mammogram was repeated and showed heterogeneous dense fibroglandular tissue and a 3.6-cm, circumscribed mass in the upper outer quadrant of the right breast (Figure 3). The mass underwent a biopsy and pathologic analysis confirmed a fibroadenoma. The patient was referred to genetic counseling given her young age. Her family history was remarkable only for a maternal grandmother (smoker) with a diagnosis of lung cancer and a paternal grandfather (smoker) in whom head and neck cancer had been diagnosed. She, however, did not follow-up for genetic testing.

Figure 1.

Figure 1

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Cross-sectional view of positron emission tomography scan showing multiple right iliac lymph nodes with mild to moderate activity (arrow).

Figure 2.

Figure 2

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Cross-sectional view of positron emission tomography scan showing a 1-cm lytic lesion at the first thoracic vertebral body with moderate activity (arrow).

Figure 3.

Figure 3

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Mammogram showing a 3.6-cm, circumscribed mass in the upper outer quadrant of the right breast (arrow), which on biopsy proved to be a fibroadenoma.

The multidisciplinary team decided to treat her disease as a stage IV breast cancer with metastasis to the bone. Palliative treatment with ovarian suppression, aromatase inhibitor, and cyclin-dependent kinase (CDK) 4/6 inhibitor was recommended; however, she declined this treatment regimen. As an alternative, tamoxifen was recommended, but the patient decided against any form of therapy at that time.

Follow-up and Outcomes

The patient did not return for follow-up until 4 months later, when she had bilateral malignant pleural effusions. The patient was started on tamoxifen, which was later stopped because of progression of disease. She refused any type of chemotherapy. She was started on a regimen of goserelin for ovarian suppression, an aromatase inhibitor (letrozole), and the CDK4/6 inhibitor palbociclib. Palbociclib was later changed to abemaciclib because of difficulty swallowing capsules. She received CDK4/6 for 6 months with stable metastatic disease on CT scan (Table 1). She also received zoledronic acid to reduce the incidence of skeletal-related events. During her course of treatments, she was admitted multiple times to the hospital because of acute respiratory failure secondary to malignant pleural effusions, and she received thoracentesis and PleurX catheter placement. During her last admission, she was placed into hospice care and died. Table 2 presents a timeline of the case.

Table 1.

Summary of clinical trials of CDK4/6 inhibitors

Trial Design Treatment arms N Media in PFS (mo) HR (95% CI) Objective response rate, % Clinical benefit rate (ITT), %
PALOMA-11 Phase II open-label Letrozole + palbociclib vs letrozole + placebo 165 20.2 vs 10.2 (treatment vs control) 0.49 (0.32–0.75) 55 vs 39 81 vs 58
PALOMA-22 Phase III placebo control Letrozole + palbociclib vs letrozole + placebo 666 24.8 vs 14.5 0.58 (0.46–0.72) 55 vs 44 85 vs 70
MONALEESA-23 Phase III placebo control Letrozole + ribociclib vs letrozole + placebo 668 25.3 vs 16 0.56 (0.43–0.72) 53 vs 37 80 vs 73
MONARCH-34 Phase III placebo control NSAI + abemaciclib vs NSAI + placebo 493 Not reached vs 14.7 0.54 (0.41–0.72) 59 vs 44 78 vs 71.5
MONALEESA-75 Phase III placebo control Tamoxifen/NSAI + goserelin + ribociclib vs tamoxifen/NSAI + goserelin + placebo 672 23.8 vs 13 0.55 (0.44–0.69) 51 vs 36 79 vs 70
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+ = plus; CDK = cyclin-dependent kinase; CI = confidence interval; HR = hazard ratio; ITT = intention to treat; MONALEESA-2 = Ribociclib plus Letrozole versus Letrozole Alone in Patients with de Novo HR+, HER2− Advanced Breast Cancer; MONALEESA-7 = Ribociclib plus Endocrine Therapy for Premenopausal Women with Hormone-Receptor-Positive, Advanced Breast Cancer; MONARCH-3 = Abemaciclib as Initial Therapy for Advanced Breast Cancer; NSAI = nonsteroidal aromatase inhibitor; PALOMA-1 = Study of Letrozole with or without Palbociclib for the First-Line Treatment of Hormone-Receptor Positive Advanced Breast Cancer; PALOMA-2 = Palbociclib + Letrozole vs. Letrozole for First-Line Treatment of Postmenopausal Women with ER+/HER2− Advanced Breast Cancer; PFS = progression-free survival.

References

1

Finn RS, Crown JP, Lang I, et al. The cyclin-dependent kinase 4/6 inhibitor palbociclib in combination with letrozole versus letrozole alone as first-line treatment of estrogen receptor-positive, HER2-negative, advanced breast cancer (PALOMA-1/TRIO-18): A randomised phase 2 study. Lancet Oncol 2015;16(1):25–35. DOI: https://doi.org/10.1016/s1470-2045(14)71159-3 PMID:25524798

2

Finn RS, Martin M, Rugo HS, et al. Palbociclib and letrozole in advanced breast cancer. N Engl J Med 2016;375(20):1925–36. DOI: https://doi.org/10.1056/nejmoa1607303 PMID:27959613

3

Hortobagyi GN, Stemmer SM, Burris HA, et al. Ribociclib as first-line therapy for HR-positive, advanced breast cancer. N Engl J Med 2016;375:1738–48. DOI: https://doi.org/10.1056/nejmx180043 PMID:27717303

4

Goetz MP, Toi M, Campone M, et al. MONARCH 3: Abemaciclib as initial therapy for advanced breast cancer. J Clin Oncol 2017;35:3638–46. DOI: https://doi.org/10.1200/jco.2017.75.6155 PMID:28968163

5

Tripathy D, et al. Ribociclib plus endocrine therapy for premenopausal women with hormone-receptor-positive, advanced breast cancer (MONALEESA-7): A randomised phase 3 trial. Lancet Oncol 2018;19(7):904–15. DOI: https://doi.org/10.1016/s1470-2045(18)30292-4 PMID:29804902

Table 2.

Timeline of the case

Relevant medical history and interventionsA 47-year-old premenopausal woman had excision of right labium majus lesion at an outside facility, with pathologic findings showing adenocarcinoma, suggestive of mammary gland type.
Date Summary and diagnostic testing Interventions
1/2018 Patient presented to Gynecologic Oncology Department
3/2018 Patient underwent radical vulvectomy and bilateral dissection of groin
4/2018 Patient presented to medical oncology clinic for initial visit. Her PET-CT showed a T1 lesion and multiple enlarged lymph nodes in groin
5/2018 Decision made in clinic to start treatment with tamoxifen Tamoxifen was started
9/26/2018 Management of bilateral malignant pleural effusions Right-sided PleurX catheter was placed
10/12/2018 Patient underwent thoracentesis
10/25/2018 Patient underwent thoracentesis
11/16/2018 Management of bilateral malignant pleural effusions Patient underwent left pleurodesis
1/2019 CT scan and bone scan showed progression of disease in bones with large unchanged bilateral loculated effusions Tamoxifen was stopped. Patient refused chemotherapy. Treatment was started with palbociclib (first filled 2/15/19), letrozole (first filled 2/29/19), and goserelin. However, patient was very noncompliant. Palbociclib changed to abemaciclib because of trouble swallowing capsules (abemaciclib first filled 5/3/19)
3/5/2019 Patient underwent thoracentesis
4/5/2019 Patient underwent thoracentesis
7/5/2019 Patient underwent thoracentesis
7/29/2019 Patient admitted for acute hypoxic respiratory failure. Repeat CT showed stable disease with large unchanged bilateral loculated effusions
7/31/2019 Patient underwent thoracentesis with insertion of a thoracostomy tube
8/20/2019 Admitted again because of respiratory failure. Patient underwent thoracentesis with no improvement. She was placed into hospice care
8/24/2019 Patient died
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CT = computed tomography; PET = positron emission tomography.

DISCUSSION

The primitive embryonic milk line extends from the axilla to the groin, including the vulva. Ectopic (accessory) breast tissue can develop if the involution of the mammary ridge is incomplete.4 The incidence of ectopic mammary gland tissue is 1% to 6% of the general population.1 Ectopic breast tissue can be found along the milk line as extranumerary nipples, extranumerary nipple areola complexes, and extranumerary breasts. The most common location of ectopic breast tissue is the axilla, followed by the area immediately inferior to the normal breast. In addition to these areas, its location in the acromial or scapular region, the vulva, and the midline of the thorax and abdomen has also been rarely reported. In 1872, Hartung reportedly presented the first case of a fully formed mammary gland in the left labium majus of a 30-year-old woman.5–8

Ectopic primary breast cancer of the vulva is rare. The first case of ectopic breast tissues in the vulva was reported by Greene9 in 1936. Since then, an additional 36 cases of primary ectopic breast cancers have been described in the literature (Table 3).1,5,10–42 Any of the various histologic subtypes of breast cancer may occur in the vulva, including infiltrating ductal, lobular, mucinous, and mixed ductal and lobular carcinomas.33,43

Table 3.

Characteristics of reported cases of primary ectopic breast cancer of the vulvaa

Case no. Author, y Age, y Clinical features Histologic type Receptors Lymph node biopsy Surgery RT/chemo/endocrine therapy Outcome, mo
1 Greene,9 1936 49 20 cm × 15-cm mass, right labium majus Ductal Not done Not done None None DOD (1)
2 Hendrix, Behrman10 1956 58 3-cm nodule, left labium minus Ductal Not done Not done Radical vulvectomy None NA
3 Guerry, Pratt-Thomas11 1976 52 1.5-cm nodule, left labium majus Ductal Not done Not done WLE None DOD (24)
4 Guercio, et al12 1984 49 2-cm cyst, left labium majus Lobular Not done 11/24 Radical vulvectomy, bilateral LND RT Alive (36)
5 Cho, et al13 1985 70 3 cm × 4 cm × 4-cm nodule, right labium majus Ductal ER+, PR+ 2/9 Radical right hemivulvectomy, ipsilateral LND Tamoxifen Alive (24)
6 Simon, et al5 1988 60 2 cm × 2-cm nodule, right labium majus Ductal ER+, PR+ 3/11 Radical vulvectomy, bilateral LND RT, chemo, tamoxifen DOD (27)
7 Rose, et al14 1990 68 3.5 cm × 3.5-cm mass, right labium majus Mixed ductal and lobular features ER+, PR+ 1/15 Radical vulvectomy + ipsilateral LND RT, tamoxifen NA
8 Di Bonito, et al15 1992 42 1.5-cm nodule, right labium majus Ductal Not done 11/13 Radical vulvectomy, bilateral LND None Alive (4)
9 Bailey, et al16 1993 65 3 cm × 2-cm lesion, right labium majus Ductal ER+, PR+ 2/20 Radical vulvectomy, bilateral LND Tamoxifen Alive (12)
10 Levin, et al17 1995 62 2.5-cm nodule, left periclitoral region Ductal ER+, PR−, HER2/neu+ 4/11 WLE, ipsilateral LND Tamoxifen Bone mets (24) Alive (36)
11 Kennedy, et al18 1997 71 5 cm × 2-cm mass, left labium majus Ductal ER−, PR− 9/9 Radical vulvectomy, bilateral LND Chemo, RT Alive (5)
12 Erb-Gremillet, et al19 1999 62 NA Adeno NA NA NA NA NA
13 Irvin,et al20 1999 64 2.7 cm × 1-cm nodule, left mons pubis Mixed ductal and lobular ER+, PR+ 1/14 WLE, ipsilateral LND Chemo, RT, tamoxifen Alive (36)
14 Gorisek, et al21 2000 81 2 cm × 3-cm mass, left labium majus Ductal ER+, PR+ NA WLE Tamoxifen Alive (19)
15 Neumann, et al22 2000 60 Tumor, right labium majus Lobular ER+, PR+ 21/21 WLE RT, chemo, tamoxifen Alive (20)
16 Piura, et al23 2002 69 3 cm × 3 cm × 3-cm nodule, left labium majus Ductal ER+, PR+ 7/15 Radical vulvectomy, bilateral LND RT, chemo, tamoxifen Alive (12)
17 Chung-Park, et al24 2002 47 2-cm nodule, right labium minus Mucinous ER+, PR−, HER2− NA Radical vulvectomy None Alive (36)
18 Yin, et al25 2003 84 5 cm, left mons pubis Mucinous ER+, PR−, HER2− 1/11 Partial vulvectomy, bilateral LND None Alive (9)
19 Ohira, et al26 2004 82 5 cm × 3-cm mass, left labium majus Ductal (comedo) ER+ Lymph node+ WLE, ipsilateral LND NA Alive (20)
20 Tanaka, et al27 2005 87 4 cm, left labium majus Adeno ER−, PR−, HER2 status? NA No surgery performed Chemo with paclitaxel for 20 wk, which responded to treatment, but patient refused further treatment Bone scan + for mets in sternum, costa, and vertebrae. Refused follow-up.
21 Lopes, et al28 2006 44 2 cm Mucinous ER+, PR status?, HER2/neu- 2/13 Partial LND CT, tamoxifen NA
22 Intra, et al29 2006 53 2-cm nodule, left labium majus Ductal ER+, PR+, HER2− 0/2 SLN WLE, SLN biopsy Tamoxifen Alive (20)
23 Fracchioli, et al30 2006 57 1 cm, left vulvar mass Adeno ER−, PR− 7/7 LN Vulvectomy CT Mets (36)
24 Abbot, et al31 2006 51 1 cm, right labium majus Adeno ER−, PR−, HER2− NA Local excision followed by Mohs surgery NA Alive (26)
25 Martinez-Palones, et al32 2007 49 3.5 cm × 3.0-cm, right labium majus Ductal ER+, PR+ 0/14 WLE, ipsilateral LND Tamoxifen NA
26 North, et al33 2007 49 1.5 cm, right of clitoris involving labia minora Adeno ER+, PR+, HER2− 5/7 WLE, left LND Chemo, RT, tamoxifen NA
27 Tseung, et al34 2008 49 Right labium majus near clitoral hood Ductal ER+, PR+ LN+ NA NA NA
28 Naseer, et al35 2011 57 1.5 cm, right labium majus Ductal ER+, PR+, HER2− 3/13 WLE, bilateral LND Chemo, RT, AI Alive (NA)
29 Diniz da Costa, et al36 2012 82 2 cm, left labia minus Adeno ER+, PR+ NA Local excision RT, AI Alive (48)
30 McMaster, et al37 2013 60 3 cm, left labium majus Adeno ER+ NA WLE RT Alive (1)
31 Bogani, et al38 2013 71 4 cm, left labium majus Ductal ER+, PR+ 1/8 Radical vulvectomy, LND Chemo, tamoxifen Alive (24)
32 Lamb, et al39 2013 59 1 cm, left labium majus Adeno ER+, PR+, HER2− Lymph node- Radical local excision, LND Tamoxifen NA
33 Benito, et al40 2013 82 4 cm, left labium Ductal ER+, PR+, HER2− 27/32 Radical vulvectomy, bilateral LND AI Alive (24)
34 Cripe, et al41 2015 62 1.3 cm, left Ductal ER+, PR+, HER2− 0/14 WLE, LND None Recurrence. Repeated WLE and LND with 3 lymph nodes+ followed by CT, RT, and AI.
35 Ishigaki,et al42 2017 72 Right side of vulva Ductal ER+, PR+, HER2− 0/1 SLN WLE, SLN biopsy AI Alive (6)
36 Eom, et al1 2017 70 1.7 cm, perineum Ductal ER+, PR+, HER2− 2 lymph nodes+ WLE, bilateral LND Chemo, AI Alive (8)
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a

The patient in case no. 36 had ectopic male breast cancer in the perineum.

Adeno = adenocarcinoma; AI = aromatase inhibitor; chemo = chemotherapy; DOD = dead of disease; ER = estrogen receptor; HER2 = human epidermal growth factor receptor 2; LND = lymph node dissection; mets = metastasis; NA = not available; PR = progesterone receptor; RT = radiation therapy; SLN = sentinel lymph node; WLE = wide local excision; + = positive; − = negative; ? = unknown.

An alternative theory, other than the incomplete involution of the mammary ridges, exists to explain the presence of ectopic breast tissue in the vulva.27 van der Putte44 described a variant of cutaneous glands in the anogenital region that resembled mammary glands. Because of the similarity to breast parenchyma, these structures were called mammarylike glands of the vulva and have similar neoplastic potential. Adenocarcinoma developing from mammarylike glands is called mammarylike adenocarcinomas of the vulva. These are locally aggressive tumors that frequently recur and involve lymph node metastases in approximately 60% of cases.31

For a diagnosis of this disease, a thorough metastatic workup should be performed, with attention directed toward detecting a breast primary cancer by the results of the history, physical examination, and radiologic examination of the breasts to confirm that the vulvar lesion is the primary site as opposed to a metastasis from a primary breast cancer. The possible rare association of these lesions with synchronous or metachronous breast cancers (16 cases in the literature) makes the exclusion of breast lesions an important part of management. Irvin et al20 defined the criteria for diagnosing this disease. When an in situ component is not present, in the absence of concurrent breast carcinoma, the following should be sufficient to categorize the lesion of primary vulvar origin: 1) a morphologic pattern consistent with breast carcinoma; 2) the presence of estrogen and progesterone receptors; and/or 3) positivity for common breast cancer markers such as epithelial membrane antigen, carcinoembryonic antigen, and glandular keratins. On the basis of these criteria, we diagnosed this disease as an adenocarcinoma of mammarylike glands in the vulva.

In most cases, surgical treatment of primary ectopic breast cancer of the vulva includes surgical excision with inguinal lymph node dissection. Sentinel lymph node biopsy could be considered as an effective alternative to inguinal node dissection.34 In a randomized controlled clinical trial of patients with vulvar cancer with inguinal node metastasis, postoperative pelvic and inguinal radiation therapy showed significant recurrence-free and cancer-related death benefit compared with ipsilateral pelvic node resection after radical vulvectomy and inguinal lymphadenectomy.45

Because of the rarity of the diagnosis and the lack of definitive treatment guidelines, this type of cancer is currently staged and treated according to current Tumor, Node, Metastasis (TNM)-based classification applicable to primary breast cancer. Therefore, treatment should consist of an individualized combination of surgery, chemotherapy, monoclonal antibody therapy, radiation, and adjuvant endocrine therapy, as appropriate.

The combination of a CDK4/6 inhibitor with endocrine therapy represents the standard first-line treatment of metastatic hormone receptor-positive breast cancer, for both premenopausal (like the patient described in our case report) and postmenopausal women. Dysregulated cellular proliferation is considered one of the “hallmarks” of cancer, and in many breast cancer cells, tumor proliferation is related to hyperactivity of the cyclin D-CDK4/6 axis, making pharmacologic blockade of this axis an attractive therapeutic strategy.10,11,46 Three CDK4/6 inhibitors have now been approved by the US Food and Drug Administration for the treatment of estrogen receptor-positive metastatic breast cancer: palbociclib (PD0332991), ribociclib (LEE011), and abemaciclib (LY835219). The addition of these agents to endocrine therapy has resulted in significant improvement of progression-free survival (Table 3). Of note, the results of the phase III MONALEESA-7 trial showed that the efficacy of the CDK4/6 inhibitor ribociclib in premenopausal or perimenopausal women when they receive ovarian function suppression together with oral endocrine therapy (tamoxifen or an aromatase inhibitor) is very similar to that observed in the postmenopausal trials (Table 1).46 This was the first major study to include tamoxifen as one of the endocrine therapy partners to CDK4/6 inhibition, and a similar improvement in progression-free survival was observed with either endocrine regimen.46

CONCLUSION

Primary breast cancer, arising from embryonic mammary ridge remnants, is an extremely rare histologic subtype of vulvar cancer. Diagnosis rests on the pathologic findings, with recognition of the characteristic histologic features and the presence of estrogen and/or progesterone receptors after the possibility of metastatic disease from breast primary cancer has been ruled out. Therapy should consist of an individualized combination of surgery, radiotherapy, chemotherapy, antiestrogen therapy, and monoclonal antibody therapy, similar to cancer of the orthotopic breast of similar stage. Owing to the rarity of this lesion, clinical trials to determine optimum treatment are not available, and management guidelines will rely on small series or case studies.

Acknowledgments

Kathleen Louden, ELS, of Louden Health Communications performed a primary copy edit.

Footnotes

Disclosure Statement

The author(s) have no conflicts of interest to disclose.

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