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Synchronous vulvar and breast cancer
European Journal of Obstetrics & Gynecology and Reproductive Biology 100 (2001) 92±93
Case report
Synchronous vulvar and breast cancer Antoine Abu-Musaa,*, Ali Khalila, Ghina Ghaziria, Muhheidine Seouda, Jaber Abbasb a
Department of Obstetrics and Gynecology, American University of Beirut Medical Center, P.O. Box 113-6044-6A, Beirut, Lebanon b Department of Surgery, American University of Beirut Medical Center, Beirut, Lebanon Received 17 October 2000; accepted 14 June 2001
Abstract Synchronous vulvar and breast cancer is rare. A 44-year-old women presented with a lesion in the right labia majora and right upper quadrant breast lump. After work-up, she underwent radical wide local vulvar excision and modi®ed radical mastectomy with axillary lymph node dissection. The pathology of the vulva revealed moderately-differentiated squamous cell carcinoma and that of the breast in®ltrating ductal carcinoma. Only two such cases have been previously reported: one was an elderly patient and the second a young patient with HIV infection. Our patient is a young and healthy woman making her presentation a unique and rare case. # 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Vulvar cancer; Breast cancer; Synchronous cancers
1. Introduction The synchronous occurrence of multiple primary malignant neoplasms in different tissues is becoming more widely recognized. The reported incidence of synchronous multiple neoplasms of the genital organs and breast is approximately 7% [1]. It is well recognized that a signi®cant number of patients with vulvar neoplasia have previous, concurrent or subsequent genital tract neoplasia. However, the occurrence of simultaneous and independent primary malignancy in the vulva and breast is an extremely rare entity. Such a case along with literature review is presented. 2. Case report A 44-year-old G1 P1 middle class housewife presented to the gynecology clinic at the American University of Beirut Medical Center, complaining of vulvar itching of 3 months duration. Pelvic examination revealed a 1 cm 1 cm eczematoid lesion in the right labia majora. Inguinal lymph nodes were not palpable. Physical examination also revealed a ®xed and hard 3 cm right upper quadrant lump in the left breast. She had no palpable axillary lymph nodes and no nipple discharge. Biopsy of the vulvar lesion revealed
* Corresponding author. Tel.: 961-1-350000; fax: 961-1-744464. E-mail address: [email protected] (A. Abu-Musa).
moderately-differentiated in®ltrating squamous cell carcinoma. A subsequent ®ne needle aspiration of the breast lump demonstrated in®ltrating ductal carcinoma with intermediate nuclear and histologic grades. The patient had no medical problems and is not maintained on any medications. She denied any history of hereditary cancers or combination of tumors. The patient's work-up included a normal chest X-ray, non-revealing CT scan of abdomen and pelvis, normal bone scan, normal blood studies including HIV titers and a negative pap smear. She underwent radical wide local excision of the vulvar lesion and left modi®ed radical mastectomy with axillary lymph node dissection. Inguinal lymphadenectomy was not performed because the breast surgery was prolonged for technical reasons. The ®nal pathology of the vulva revealed a 1 cm 0:5 cm moderately-differentiated squamous cell carcinoma with negative margins and depth of invasion <2 mm. Pathology of the breast revealed a 2.8 cm in®ltrating ductal carcinoma grade 2±3 and free margins. Metastasis to 3 out of the 12 axillary lymph nodes was noted. Estrogen receptors were negative while progesterone receptors were positive. The patient had a smooth and uncomplicated post-operative course and has no evidence of recurrent vulvar cancer. As for breast cancer, she received adjuvant chemotherapy (cyclophosphamide, adriamycin, 5-¯ourouracil) and left chest wall radiotherapy. So far, she has no evidence of recurrent or metastatic disease with normal CA 15-3 and mammogram of the contralateral breast. The patient is maintained on tamoxifen 20 mg daily.
0301-2115/01/$ ± see front matter # 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 3 0 1 - 2 1 1 5 ( 0 1 ) 0 0 4 6 1 - 4
A. Abu-Musa et al. / European Journal of Obstetrics & Gynecology and Reproductive Biology 100 (2001) 92±93
3. Comments Patients who have one malignancy are at risk of developing another cancer. Billroth, in 1869, was the ®rst to report the presence of different primary malignant tumors in the same patient. The incidence of multiple primary tumors among the total population of cancer is estimated to be 2.5% [2]. The etiology of multiple primary neoplasms is unknown. Suggested factors include: genetic, hormonal, iatrogenic, environmental and immunological causes. Warren and Gates established three criteria for the diagnosis of multiple primary malignancies [3]. These include (1) each tumor should be distinct; (2) present a de®nitive picture of malignancy; and (3) the probability that one tumor is a metastatic lesion from the other must be excluded. Vulvar neoplasia have been associated with other primary malignancies [4]. Most of these malignancies are anogenital cancers with cervical cancer being the most frequent other primary cancer. These results suggest that vulvar cancer associated with other primary malignancies of the genital tract may have a common etiology, in particular, an infectious element [4]. The association of genital organs neoplasm and breast cancer is well-known. The majority of patients present with ovarian or endometrial cancer. Almost all reported cases of simultaneous occurrence of vulvar and breast cancer have been limited to Paget's disease [5] and mammary cancer [6]. However, the synchronous presentation of completely distinct vulvar and breast cancer is an
93
extremely rare entity. To our knowledge, only two cases have been reported in the literature. The ®rst patient was a 72-year-old lady with breast, colon and vulvar cancer [7]. The second case was reported in a young patient with HIV infection [8]. It is known that the risk of multiple primary neoplasms increases with age and in immunode®cient patients. Our patient is a young and healthy woman with no HIV infection making her presentation a unique and rare case. References [1] Deligdisch L, Szulman EE. Multiple and multifocal carcinomas in female genital organs and breast. Gynecol Oncol 1975;3: 181±90. [2] Gil Extremera B. Incidence of multiple primary malignancies. A retrospective study of 10 years. Rev Esp Oncol 1980;27:251±63. [3] Warren S, Gates O. Multiple primary tumors: a survey of the literature and a statistical study. Am J Cancer 1932;16:1358. [4] Sherman KJ, Daling JR, Chu J, McKnight B, Weiss NS. Multiple primary tumours in women with vulvar neoplasms: a case control study. Br J Cancer 1988;57:423±7. [5] Popiolek DA, Hajdu SI, Gal D. Synchronous Paget's disease of the vulva and breast. Gynecol Oncol 1998;71:137±40. [6] Guerry RL, Pratt-Thomas HR. Carcinoma of the supernumerary breast of vulva with bilateral mammary cancer. Cancer 1976;38:2570±4. [7] Deppe G, Dolan T, Zbella E, Heredia R. Synchronous multiple primary malignant neoplasms of the breast, colon and vulva. J Reprod Med 1984;29:878±80. [8] Rose P, Fraire A. Multiple primary gynecologic neoplasms in a young HIV-positive patient. J Surg Oncol 1993;53:269±72.
Case report
Synchronous vulvar and breast cancer Antoine Abu-Musaa,*, Ali Khalila, Ghina Ghaziria, Muhheidine Seouda, Jaber Abbasb a
Department of Obstetrics and Gynecology, American University of Beirut Medical Center, P.O. Box 113-6044-6A, Beirut, Lebanon b Department of Surgery, American University of Beirut Medical Center, Beirut, Lebanon Received 17 October 2000; accepted 14 June 2001
Abstract Synchronous vulvar and breast cancer is rare. A 44-year-old women presented with a lesion in the right labia majora and right upper quadrant breast lump. After work-up, she underwent radical wide local vulvar excision and modi®ed radical mastectomy with axillary lymph node dissection. The pathology of the vulva revealed moderately-differentiated squamous cell carcinoma and that of the breast in®ltrating ductal carcinoma. Only two such cases have been previously reported: one was an elderly patient and the second a young patient with HIV infection. Our patient is a young and healthy woman making her presentation a unique and rare case. # 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Vulvar cancer; Breast cancer; Synchronous cancers
1. Introduction The synchronous occurrence of multiple primary malignant neoplasms in different tissues is becoming more widely recognized. The reported incidence of synchronous multiple neoplasms of the genital organs and breast is approximately 7% [1]. It is well recognized that a signi®cant number of patients with vulvar neoplasia have previous, concurrent or subsequent genital tract neoplasia. However, the occurrence of simultaneous and independent primary malignancy in the vulva and breast is an extremely rare entity. Such a case along with literature review is presented. 2. Case report A 44-year-old G1 P1 middle class housewife presented to the gynecology clinic at the American University of Beirut Medical Center, complaining of vulvar itching of 3 months duration. Pelvic examination revealed a 1 cm 1 cm eczematoid lesion in the right labia majora. Inguinal lymph nodes were not palpable. Physical examination also revealed a ®xed and hard 3 cm right upper quadrant lump in the left breast. She had no palpable axillary lymph nodes and no nipple discharge. Biopsy of the vulvar lesion revealed
* Corresponding author. Tel.: 961-1-350000; fax: 961-1-744464. E-mail address: [email protected] (A. Abu-Musa).
moderately-differentiated in®ltrating squamous cell carcinoma. A subsequent ®ne needle aspiration of the breast lump demonstrated in®ltrating ductal carcinoma with intermediate nuclear and histologic grades. The patient had no medical problems and is not maintained on any medications. She denied any history of hereditary cancers or combination of tumors. The patient's work-up included a normal chest X-ray, non-revealing CT scan of abdomen and pelvis, normal bone scan, normal blood studies including HIV titers and a negative pap smear. She underwent radical wide local excision of the vulvar lesion and left modi®ed radical mastectomy with axillary lymph node dissection. Inguinal lymphadenectomy was not performed because the breast surgery was prolonged for technical reasons. The ®nal pathology of the vulva revealed a 1 cm 0:5 cm moderately-differentiated squamous cell carcinoma with negative margins and depth of invasion <2 mm. Pathology of the breast revealed a 2.8 cm in®ltrating ductal carcinoma grade 2±3 and free margins. Metastasis to 3 out of the 12 axillary lymph nodes was noted. Estrogen receptors were negative while progesterone receptors were positive. The patient had a smooth and uncomplicated post-operative course and has no evidence of recurrent vulvar cancer. As for breast cancer, she received adjuvant chemotherapy (cyclophosphamide, adriamycin, 5-¯ourouracil) and left chest wall radiotherapy. So far, she has no evidence of recurrent or metastatic disease with normal CA 15-3 and mammogram of the contralateral breast. The patient is maintained on tamoxifen 20 mg daily.
0301-2115/01/$ ± see front matter # 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 3 0 1 - 2 1 1 5 ( 0 1 ) 0 0 4 6 1 - 4
A. Abu-Musa et al. / European Journal of Obstetrics & Gynecology and Reproductive Biology 100 (2001) 92±93
3. Comments Patients who have one malignancy are at risk of developing another cancer. Billroth, in 1869, was the ®rst to report the presence of different primary malignant tumors in the same patient. The incidence of multiple primary tumors among the total population of cancer is estimated to be 2.5% [2]. The etiology of multiple primary neoplasms is unknown. Suggested factors include: genetic, hormonal, iatrogenic, environmental and immunological causes. Warren and Gates established three criteria for the diagnosis of multiple primary malignancies [3]. These include (1) each tumor should be distinct; (2) present a de®nitive picture of malignancy; and (3) the probability that one tumor is a metastatic lesion from the other must be excluded. Vulvar neoplasia have been associated with other primary malignancies [4]. Most of these malignancies are anogenital cancers with cervical cancer being the most frequent other primary cancer. These results suggest that vulvar cancer associated with other primary malignancies of the genital tract may have a common etiology, in particular, an infectious element [4]. The association of genital organs neoplasm and breast cancer is well-known. The majority of patients present with ovarian or endometrial cancer. Almost all reported cases of simultaneous occurrence of vulvar and breast cancer have been limited to Paget's disease [5] and mammary cancer [6]. However, the synchronous presentation of completely distinct vulvar and breast cancer is an
93
extremely rare entity. To our knowledge, only two cases have been reported in the literature. The ®rst patient was a 72-year-old lady with breast, colon and vulvar cancer [7]. The second case was reported in a young patient with HIV infection [8]. It is known that the risk of multiple primary neoplasms increases with age and in immunode®cient patients. Our patient is a young and healthy woman with no HIV infection making her presentation a unique and rare case. References [1] Deligdisch L, Szulman EE. Multiple and multifocal carcinomas in female genital organs and breast. Gynecol Oncol 1975;3: 181±90. [2] Gil Extremera B. Incidence of multiple primary malignancies. A retrospective study of 10 years. Rev Esp Oncol 1980;27:251±63. [3] Warren S, Gates O. Multiple primary tumors: a survey of the literature and a statistical study. Am J Cancer 1932;16:1358. [4] Sherman KJ, Daling JR, Chu J, McKnight B, Weiss NS. Multiple primary tumours in women with vulvar neoplasms: a case control study. Br J Cancer 1988;57:423±7. [5] Popiolek DA, Hajdu SI, Gal D. Synchronous Paget's disease of the vulva and breast. Gynecol Oncol 1998;71:137±40. [6] Guerry RL, Pratt-Thomas HR. Carcinoma of the supernumerary breast of vulva with bilateral mammary cancer. Cancer 1976;38:2570±4. [7] Deppe G, Dolan T, Zbella E, Heredia R. Synchronous multiple primary malignant neoplasms of the breast, colon and vulva. J Reprod Med 1984;29:878±80. [8] Rose P, Fraire A. Multiple primary gynecologic neoplasms in a young HIV-positive patient. J Surg Oncol 1993;53:269±72.