History

The majority of patients present with a single vulvar lesion. Patients may complain of vulvar pruritus, pain, or irritation, though many patients will be asymptomatic. Patients with advanced disease can present with enlarged inguinal lymph nodes, lower-extremity edema, or rectal bleeding.

Physical Examination

Patients presenting with vulvar complaints should have a complete pelvic examination, including palpation for enlarged inguinal lymph nodes, vaginal speculum examination, and bimanual pelvic examination. Biopsy any abnormal lesions suspicious for cancer. The usual appearance of a vulvar cancer is a raised red lesion, which is firm or friable depending on the size of the lesion.

Differential Diagnoses

Grigsby, PW. Role of PET in gynecologic malignancy. Current Opinion in Oncology. 09/2009. 21:420-424. [QxMD MEDLINE Link].
Basset A. Traitement chirurgical operatoire de l'epithelima primitif du clitoris indications-technique-resultats. Rev Chir. 1912. 46:546.
Way S. Carcinoma of the vulva. Am J Obstet Gynecol. 1960 Apr. 79:692-7. [QxMD MEDLINE Link].
Morley GW. Infiltrative carcinoma of the vulva: results of surgical treatment. Am J Obstet Gynecol. 1976. 124:874-878.
Taussig FJ. Cancer of the Vulva. Am J Obstet Gynecol. 1940. 40:764.
Hacker NF, Leuchter RS, Berek JS, et al. Radical vulvectomy and bilateral inguinal lymphadenectomy through separate groin incisions. Obstet Gynecol. 1981 Nov. 58(5):574-9. [QxMD MEDLINE Link].
Siegel RL, Miller KD, Fuchs HE, Jemal A. Cancer Statistics, 2021. CA Cancer J Clin. 2021 Jan. 71 (1):7-33. [QxMD MEDLINE Link]. [Full Text].
Moore DH, Thomas GM, Montana GS. Preoperative chemoradiation for advanced vulvar cancer: a phase II study of the Gynecologic Oncology Group. Int J Radiat Oncol Biol Phys. 1998 Aug 1. 42(1):79-85. [QxMD MEDLINE Link].
Iversen T, Aas M. Lymph drainage from the vulva. Gynecol Oncol. 1983 Oct. 16(2):179-89. [QxMD MEDLINE Link].
Wilkinson EJ. The 1989 presidential address. International Society for the Study of Vulvar Disease. J Reprod Med. 1990 Nov. 35(11):981-91. [QxMD MEDLINE Link].
van Doorn HC, Ansink A, Verhaar-Langereis MMJ, Stalpers LL. Neoadjuvant chemoradiation for advanced primary vulvar cancer (Review). The Cochrane Collaboration. 2009. 1:1-20. [Full Text].
Tantipalakorn C, Robertson G, Marsden DE, Gebski V, Hacker NF. Outcome and Patterns of Recurrence for International Federation of Gynecology and Obstetrics (FIGO) Stages I and II Squamous Cell Vulvar Cancer. Obstet Gynecol. April/2009. 113:895-901.
Clark WH Jr, From L, Bernardino EA, Mihm MC. The histogenesis and biologic behavior of primary human malignant melanomas of the skin. Cancer Res. 1969 Mar. 29(3):705-27. [QxMD MEDLINE Link].
Chung AF, Woodruff JM, Lewis JL Jr. Malignant melanoma of the vulva: A report of 44 cases. Obstet Gynecol. 1975 Jun. 45(6):638-46. [QxMD MEDLINE Link].
Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous melanoma. Ann Surg. 1970 Nov. 172(5):902-8. [QxMD MEDLINE Link].
FIGO Committee on Gynecologic Oncology. Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium. Int J Gynecol Obst. 2009. 105:103-104.
American Joint Committee on Cancer. AJCC Cancer Staging Manual. 7th. Chicago, Illinois: Springer New York, Inc; 2010.
Trope C, Johnsson JE, Larsson G, Simonsen E. Bleomycin alone or combined with mitomycin C in treatment of advanced or recurrent squamous cell carcinoma of the vulva. Cancer Treat Rep. 1980 Apr-May. 64(4-5):639-42. [QxMD MEDLINE Link].
Deppe G, Bruckner HW, Cohen CJ. Adriamycin treatment of advanced vulvar carcinoma. Obstet Gynecol. 1977 Jul. 50(1 Suppl):13s-14s. [QxMD MEDLINE Link].
Shylasree TS, Bryant A, Howells RE. Chemoradiation for advanced primary vulval cancer. Cochrane Database Syst Rev. 2011 Apr 13. 4:CD003752. [QxMD MEDLINE Link].
Zhang SH, Sood AK, Sorosky JI, et al. Preservation of the saphenous vein during inguinal lymphadenectomy decreases morbidity in patients with carcinoma of the vulva. Cancer. 2000 Oct 1. 89(7):1520-5. [QxMD MEDLINE Link].
Rouzier R, Haddad B, Dubernard G. Inguinofemoral dissection for carcinoma of the vulva: effect of modifications of extent and technique on morbidity and survival. J Am Coll Surg. 2003 Mar. 196(3):442-50. [QxMD MEDLINE Link].
Ansink A, Stegemen M, van der Velden K, Collingwood M. Surgical interventions for early squamous cell carcinoma of the vulva (Review). The Cochran Collaboration. 2008. 3:1-20. [Full Text].
Homesley HD, Bundy BN, Sedlis A, Adcock L. Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstet Gynecol. 1986 Dec. 68(6):733-40. [QxMD MEDLINE Link].
van der Velden K, Ansink A. Primary groin irradiation versus primary groin surgery for early vulvar cancer (Review). The Cochrane Collection. 2009. 1:1-15. [Full Text].
de Hullu JA, Hollema H, Piers DA, Verheijen RH, van Diest PJ, Mourits MJ, et al. Sentinel lymph node procedure is highly accurate in squamous cell carcinoma of the vulva. J Clin Oncol. 2000 Aug. 18(15):2811-6. [QxMD MEDLINE Link].
Martínez-Palones JM, Pérez-Benavente MA, Gil-Moreno A, Díaz-Feijoo B, Roca I, García-Jiménez A. Comparison of recurrence after vulvectomy and lymphadenectomy with and without sentinel node biopsy in early stage vulvar cancer. Gynecol Oncol. 2006 Dec. 103(3):865-70. [QxMD MEDLINE Link].
Ate GJ, Van der Zee, Oonk MH, De Hulla JA, Ansink AC, Vergote I, et al. Sentinel node dissection is safe in the treatment of early-stage vulvar cancer. J Clin Oncol. Feb 2008. 26:884-89. [Full Text].
Levenback CF, Ali S, Coleman RL, et al. Lymphatic mapping and sentinel lymph node biopsy in women with squamous cell carcinoma of the vulva: a gynecologic oncology group study. J Clin Oncol. 2012 Nov 1. 30(31):3786-91. [QxMD MEDLINE Link]. [Full Text].
Hassanzade M, Attaran M, Treglia G, Yousefi Z, Sadeghi R. Lymphatic mapping and sentinel node biopsy in squamous cell carcinoma of the vulva: systematic review and meta-analysis of the literature. Gynecol Oncol. 2013 Jul. 130(1):237-45. [QxMD MEDLINE Link].
Yoder BJ, Rufforny I, Massoll NA, Wilkinson EJ. Stage IA vulvar squamous cell carcinoma: an analysis of tumor invasive characteristics and risk. Am J Surg Pathol. 2008 May. 32(5):765-72. [QxMD MEDLINE Link].
Helm CW, Hatch K, Austin JM, et al. A matched comparison of single and triple incision techniques for the surgical treatment of carcinoma of the vulva. Gynecol Oncol. 1992 Aug. 46(2):150-6. [QxMD MEDLINE Link].
Judson PL, Jonson AL, Paley PJ, Bliss RL, Murray KP, Downs LS Jr, et al. A prospective, randomized study analyzing sartorius transposition following inguinal-femoral lymphadenectomy. Gynecol Oncol. 2004 Oct. 95(1):226-30. [QxMD MEDLINE Link].
Berman ML, Soper JT, Creasman WT, et al. Conservative surgical management of superficially invasive stage I vulvar carcinoma. Gynecol Oncol. 1989 Dec. 35(3):352-7. [QxMD MEDLINE Link].
Stehman FB, Bundy BN, Dvoretsky PM, Creasman WT. Early stage I carcinoma of the vulva treated with ipsilateral superficial inguinal lymphadenectomy and modified radical hemivulvectomy: a prospective study of the Gynecologic Oncology Group. Obstet Gynecol. 1992 Apr. 79(4):490-7. [QxMD MEDLINE Link].
Kirby TO, Rocconi RP, Numnum TM, Kendrick JE, Wright J, Fowler W, et al. Outcomes of Stage I/II vulvar cancer patients after negative superficial inguinal lymphadenectomy. Gynecol Oncol. 2005 Aug. 98(2):309-12. [QxMD MEDLINE Link].
Levenback C, Morris M, Burke TW, et al. Groin dissection practices among gynecologic oncologists treating early vulvar cancer. Gynecol Oncol. 1996 Jul. 62(1):73-7. [QxMD MEDLINE Link].
Aerts L, Enzlin P, Vergote I, Verhaeghe J, Poppe W, Amant F. Sexual, psychological, and relational functioning in women after surgical treatment for vulvar malignancy: a literature review. J Sex Med. 2012 Feb. 9(2):361-71. [QxMD MEDLINE Link].
[Guideline] Kunos C, Simpkins F, Gibbons H, Tian C, Homesly H. Radiation therapy compared with pelvic node resection for node-positive vulvar cancer. Obstet Gynecol. September/2009. 114:537-46.
Mahner, S, Trillsch F, Kock L, Rohsbach, D, et al. Adjuvant therapy in node-positive vulvar cancer. Expert Review of Anticancer Therapy. 2013. 13:7:839-844. [QxMD MEDLINE Link]. [Full Text].
[Guideline] NCCN Clinical Practice Guidelines in Oncology. Vulvar Cancer (Squamous Cell Carcinoma). NCCN.org. Available at http://www.nccn.org/professionals/physician_gls/pdf/vulvar.pdf. 01/14/16; Accessed: April 12, 2016.
Koutsky LA, Ault KA, Wheeler CM, Brown DR, Barr E, Alvarez FB. A controlled trial of a human papillomavirus type 16 vaccine. N Engl J Med. 2002 Nov 21. 347(21):1645-51. [QxMD MEDLINE Link].
Garland SM, Hernandez-Avila M, Wheeler CM, Perez G, Harper DM, Leodolter S. Quadrivalent vaccine against human papillomavirus to prevent anogenital diseases. N Engl J Med. 2007 May 10. 356(19):1928-43. [QxMD MEDLINE Link].
The FUTURE II Study Group. Quadrivalent vaccine against human papillomavirus to prevent high-grade cervical lesions. N Engl J Med. 2007 May 10. 356(19):1915-27. [QxMD MEDLINE Link].
Gardasil [package insert]. Whitehouse Station, NJ: Merck and Co. 2008. Available at [Full Text].
Gardasil 9 [package insert]. Whitehouse Station, NJ: Merck. 2015. Available at [Full Text].
Stehman FB, Look KY. Carcinoma of the vulva. Obstet Gynecol. 2006 Mar. 107(3):719-33. [QxMD MEDLINE Link].
Deppe G, Mert I, Winer IS. Management of squamous cell vulvar review: A revies. Journal of Obstetrics and Gynaecology Research. 2014 May. 40:1217-1225. [QxMD MEDLINE Link].
Mahner S, Prieske K, Grimm D, Trillsch F, Prieske S, von Amsberg, et al. Systemic treatment of vulvar cancer. Expert Rev Anticancer Ther. 2015. 15(6):629-637. [QxMD MEDLINE Link]. [Full Text].
Decesare SL, Fiorica JV, Roberts WS, et al. A pilot study utilizing intraoperative lymphoscintigraphy for identification of the sentinel lymph nodes in vulvar cancer. Gynecol Oncol. 1997 Sep. 66(3):425-8. [QxMD MEDLINE Link].
Abitbol MM. Carcinoma of the vulva: improvements in the surgical approach. Am J Obstet Gynecol. 1973 Oct 15. 117(4):483-9. [QxMD MEDLINE Link].
Burke TW, Levenback C, Coleman RL, et al. Surgical therapy of T1 and T2 vulvar carcinoma: further experience with radical wide excision and selective inguinal lymphadenectomy. Gynecol Oncol. 1995 May. 57(2):215-20. [QxMD MEDLINE Link].
Christopherson W, Buchsbaum HJ, Voet R, Lifschitz S. Radical vulvectomy and bilateral groin lymphadenectomy utilizing separate groin incisions: report of a case with recurrence in the intervening skin bridge. Gynecol Oncol. 1985 Jun. 21(2):247-51. [QxMD MEDLINE Link].
Gonzalez Bosquet J, Magrina JF, Gaffey TA, Hernandez JL, Webb MJ, Cliby WA, et al. Long-term survival and disease recurrence in patients with primary squamous cell carcinoma of the vulva. Gynecol Oncol. 2005 Jun. 97(3):828-33. [QxMD MEDLINE Link].
Green MS, Naumann RW, Elliot M, et al. Sexual dysfunction following vulvectomy. Gynecol Oncol. 2000 Apr. 77(1):73-7. [QxMD MEDLINE Link].
Hacker NF, Berek JS, Lagasse LD. Microinvasive carcinoma of the vulva. Obstet Gynecol. 1983 Jul. 62(1):134-5. [QxMD MEDLINE Link].
Hacker NF, Berek JS, Lagasse LD, et al. Individualization of treatment for stage I squamous cell vulvar carcinoma. Obstet Gynecol. 1984 Feb. 63(2):155-62. [QxMD MEDLINE Link].
Heaps JM, Fu YS, Montz FJ, et al. Surgical-pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva. Gynecol Oncol. 1990 Sep. 38(3):309-14. [QxMD MEDLINE Link].
Klemm P, Marnitz S, Köhler C, Braig U, Schneider A. Clinical implication of laparoscopic pelvic lymphadenectomy in patients with vulvar cancer and positive groin nodes. Gynecol Oncol. 2005 Oct. 99(1):101-5. [QxMD MEDLINE Link].
Leminen A, Forss M, Paavonen J. Wound complications in patients with carcinoma of the vulva. Comparison between radical and modified vulvectomies. Eur J Obstet Gynecol Reprod Biol. 2000 Dec. 93(2):193-7. [QxMD MEDLINE Link].
Menczer J, Fintsi Y, Arbel-Alon S. The presence of HPV 16, 18 and p53 immunohistochemical staining in tumor tissue of Israeli Jewish women with cervical and vulvar neoplasia. Eur J Gynaecol Oncol. 2000. 21(1):30-4. [QxMD MEDLINE Link].
Miller B, Morris M, Levenback C, et al. Pelvic exenteration for primary and recurrent vulvar cancer. Gynecol Oncol. 1995 Aug. 58(2):202-5. [QxMD MEDLINE Link].
Schulz MJ, Penalver M. Recurrent vulvar carcinoma in the intervening tissue bridge in early invasive stage I disease treated by radical vulvectomy and bilateral groin dissection through separate incisions. Gynecol Oncol. 1989 Dec. 35(3):383-6. [QxMD MEDLINE Link].
Sedlis A, Homesley H, Bundy BN, et al. Positive groin lymph nodes in superficial squamous cell vulvar cancer. A Gynecologic Oncology Group Study. Am J Obstet Gynecol. 1987 May. 156(5):1159-64. [QxMD MEDLINE Link].
Siller BS, Alvarez RD, Conner WD, et al. T2/3 vulva cancer: a case-control study of triple incision versus en bloc radical vulvectomy and inguinal lymphadenectomy. Gynecol Oncol. 1995 Jun. 57(3):335-9. [QxMD MEDLINE Link].
Terada KY, Coel MN, Ko P, Wong JH. Combined use of intraoperative lymphatic mapping and lymphoscintigraphy in the management of squamous cell cancer of the vulva. Gynecol Oncol. 1998 Jul. 70(1):65-9. [QxMD MEDLINE Link].
Wharton JT, Gallager S, Rutledge FN. Microinvasive carcinoma of the vulva. Am J Obstet Gynecol. 1974 Jan 15. 118(2):159-62. [QxMD MEDLINE Link].
Cirik DA, Karalok A, Ureyen I, et al. Early and late complications after inguinofemoral lymphadenectomy for vulvar cancer. Asian Pac J Cancer Prev. 2015. 16(13):5175-9. [QxMD MEDLINE Link].
Nomura H, Maeda T, Usami T, et al. Vulvar reconstruction following surgery for vulvar cancer using a stepladder V-Y advancement medial thigh flap. Int J Gynecol Cancer. 2015 Oct. 25(8):1484-7. [QxMD MEDLINE Link].
Slomovitz BM, Coleman RL, Oonk MHM, van der Zee, A, Levenback C. Update on sentinel lymph node biopsy for early-stage vulvar cancer. Gynecologic Oncology. 2015. 138:472-477. [QxMD MEDLINE Link]. [Full Text].
Zweizig S, Korets S, Cain JM. Key concepts in management of vulvar cancer. Best Practice & Research Clinical Obstetrics and Gynaecology. 2014. 28:959-966. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Diagram of lymphatic drainage of a lateral lesion. Most lymphatics flow through the superficial inguinal nodes, deep inguinal nodes, and the node of Cloquet to the pelvic lymph node chains. Deep inguinal node findings are positive approximately 3% of the time when superficial inguinal node findings are negative. Lymphatic mapping studies indicate that 13% of cases demonstrate findings consistent with flow to the pelvis that does not involve the node of Cloquet. If the lesion is in the anterior labia minor, then contralateral flow is demonstrated in 67% of cases.
A large T2 carcinoma of the vulva crossing the midline and involving the clitoris. (Photograph courtesy of Tom Wilson)
Specimen after removal with at least 1 cm margins around the tumor. (Photograph courtesy of Tom Wilson)
The surgical defect after a radical vulvectomy specimen is removed. (Photograph courtesy of Tom Wilson)
The surgical defect is closed after a radical vulvectomy. (Photograph courtesy of Tom Wilson)
A large squamous cell carcinoma of the vulva. Note the small contralateral "kissing lesion" that can be seen with vulvar carcinomas. (Photograph courtesy of James B. Hall, MD)
A specimen from a traditional single-incision radical hysterectomy. Most radical vulvectomies are now performed through 3 incisions, with the groin nodes removed separately from the vulvectomy specimen. (Photograph courtesy of James B. Hall, MD)
Closure of a large single-incision radical vulvectomy. The complete wound breakdown rate from this procedure is often greater than 50%. (Photograph courtesy of James B. Hall, MD)
A specimen from a primary exenteration for a stage IVA vulvar cancer involving the rectum. Many of these large tumors are now treated with adjuvant chemotherapy and radiation prior to surgery, with preservation of rectal sphincter function. (Photograph courtesy of James B. Hall, MD)

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Table 1. Staging of vulvar cancer by FIGO (2008) and AJCC (2010)

TNM Categories	FIGO	Definition
TX		Primary tumor cannot be assessed
T0		No evidence of primary tumor
Tis*		Carcinoma in situ (preinvasive carcinoma)
T1a	IA	Lesions ≤2 cm confined to the vulva or perineum and with stromal invasion ≤1 mm†
T1b	IB	Lesions >2 cm, or any size with stromal invasion more than 1 mm, confined to the vulva or perineum
T2‡	II	Tumor of any size with extension to adjacent perineal structures (lower/distal one third of urethra, lower/distal one third of vagina, anal involvement)
T3§	IVA	Tumor of any size with extension to any of the following: upper/proximal two thirds of urethra, upper/proximal two thirds of vagina, bladder mucosa, rectal mucosa, or fixed to pelvic bone
* FIGO staging no longer includes Stage 0 (Tis). † The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion. ‡ FIGO uses the classification T2/T3. This is defined as T2 in TNM. § FIGO uses the classification T4. This is defined as T3 in TNM.

Table 2. Tumor Regional Lymph Node Involvement and Metastasis Categories and FIGO Stages

Regional lymph nodes (N)*
TNM categories	FIGO stages	Definition
NX		Regional lymph nodes cannot be assessed
N0		No regional lymph node metastasis
N1		1or 2 regional lymph nodes with the following features:
N1a	IIIA	1 lymph node metastasis each ≤5 mm
N1b	IIIA	1 lymph node metastasis ≥5 mm
N2	IIIB	Regional lymph node metastasis with the following features:
N2a	IIIB	≥3 lymph node metastases each < 5 mm
N2b	IIIB	≥2 lymph node metastases ≥5 mm
N2c	IIIC	Lymph node metastasis with extracapsular spread
N3	IVA	Fixed or ulcerated regional lymph node metastasis
Distant metastasis (M)
TNM categories	FIGO stages	Definition
M0		No distant metastasis
M1	IVB	Distant metastasis (including pelvic lymph node metastasis)
Anatomic stage/prognostic groups
Stage 0	Tis	N0	M0
Stage I	T1	N0	M0
Stage IA	T1a	N0	M0
Stage IB	T1b	N0	M0
Stage II	T2	N0	M0
Stage IIIA	T1,T2	N1a, N1b	M0
Stage IIIB	T1, T2	N2a, N2b	M0
Stage IIIC	T1, T2	N2c	M0
Stage IVA	T1, T2	N3	M0
Stage IVA	T3	Any N	M0
Stage IVB	Ant T	Any N	M1
* An effort should be made to describe the site and laterality of lymph node metastases.

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Author

Robert V Higgins, MD Professor, Department of Obstetrics/Gynecology, Division of Gynecologic Oncology, Medical College of Georgia at Augusta University

Robert V Higgins, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Society of Gynecologic Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

R Wendel Naumann, MD Clinical Assistant Professor of Obstetrics and Gynecology, University of North Carolina at Chapel Hill School of Medicine; Director, Minimally Invasive Surgery in Gynecologic Oncology, Associate Director, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Carolinas Medical Center

R Wendel Naumann, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Surgeons

Disclosure: Nothing to disclose.

James Hall, MD Director, Division of Gynecologic Oncology, Blumenthal Cancer Center, Carolinas Medical Center; Clinical Professor, Department of Obstetrics and Gynecology, University of North Carolina at Chapel Hill School of Medicine

James Hall, MD is a member of the following medical societies: American Cancer Society, Society of Gynecologic Oncology, American College of Obstetricians and Gynecologists

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Michel E Rivlin, MD Former Professor, Department of Obstetrics and Gynecology, University of Mississippi School of Medicine

Michel E Rivlin, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Medical Association, Mississippi State Medical Association, Royal College of Surgeons of Edinburgh, Royal College of Obstetricians and Gynaecologists

Disclosure: Nothing to disclose.

Chief Editor

Leslie M Randall, MD, MAS, FACS Professor and Director, Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Diane Harris Wright Professor of Gynecologic Oncology Research, Massey Cancer Center, Virginia Commonwealth University School of Medicine

Leslie M Randall, MD, MAS, FACS is a member of the following medical societies: Academy for Innovation in Medical Education, American College of Surgeons, American Medical Association, American Society of Clinical Oncology, International Gynecologic Cancer Society, Society of Gynecologic Oncology

Disclosure: Nothing to disclose.

Sections Surgical Treatment of Vulvar Cancer
Overview
Presentation
DDx
Workup
Treatment
Media Gallery
Tables
References

Surgical Treatment of Vulvar Cancer Clinical Presentation

History

Physical Examination

References

Tables

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