Assuming that our hypothesis is correct and that this was influenza A hepatitis, the following question remains: what are the implications to the fetus in this condition? Of note, influenza in pregnancy is known to put the mother at risk for pneumonia and even death, and any infectious process in pregnancy can increase the risk for premature contractions or premature birth. Another risk to consider is that of increased bile acids, which are thought to be related to the cause of sudden fetal death in cholestasis of pregnancy.1 Our patient’s bile acids increased considerably during the course of her illness, and yet the fetus survived. Obviously, based on this single case, we cannot estimate the risks that elevated bile acids would be valid in other similar cases. In summary, inflammation of the liver by influenza A infection is a T-cell–mediated process. Because it is well-known that pregnancy suppresses normal T-cell function, a process that allows maternal-fetal tolerance, it follows that influenza A hepatitis in pregnancy should be an extremely rare event. This case demonstrates that influenza A infection can be associated with hepatitis and cholestasis in pregnancy, the implications of which may be dif-
ferent from these liver disorders in other settings. Influenza-mediated hepatitis would be expected to resolve spontaneously with supportive and symptomatic care over 10 to 14 days with favorable maternal and perinatal outcomes.
Myxoid Leiomyosarcoma of the Bartholin Gland
CASE: Our patient presented with a 10 cm mass on her vulva, which was presumed to be a Bartholin gland cyst. Pathology showed a high-grade myxoid leiomyosarcoma with positive margins. She was treated with left radical hemivulvectomy, laparoscopic hysterectomy with bilateral salpingo-oophorectomy, and six cycles of adjuvant chemotherapy. She is now 1 year from completion of therapy and remains disease-free.
Erika L. Mowers, MD, Jessica J. Shank, MD, Nora Frisch, MD, and R. Kevin Reynolds,
MD, FACS
BACKGROUND: Myxoid leiomyosarcomas originating from the Bartholin gland are exceedingly rare. Only one other case of a Bartholin gland myxoid leiomyosarcoma has been reported.
REFERENCES 1. Creasy RK, Resnik R, Iams JD, Lockwood CJ, Moore TR. Creasy & Resnik’s maternal-fetal medicine: principles and practice. 6th ed. Philadelphia (PA): Saunders Elsevier; 2009. 2. Lee NM, Brady CW. Liver disease in pregnancy. World J Gastroenterol 2009;15:897–906. 3. Adams DH, Hubscher SG. Commentary: systemic viral infections and collateral damage in the liver. Am J Pathol 2006;168: 1057–9. 4. Basir N, Yew TG, Telisinghe PU, Chong VH. Autoimmune hepatitis in children: a report of two cases. Singapore Med J 2011;52:e27–30. 5. Papic N, Pangercic A, Vargovic M, Barsic B, Vince A, Kuzman I. Liver involvement during influenza infection: perspective on the 2009 influenza pandemic. Influenza Other Respir Viruses 2012;6:e2–5. 6. Polakos NK, Cornejo JC, Murray DA, Write KO, Treanor JJ, Crispe IN. Kupffer cell-dependent hepatitis occurs during influenza infection. Am J Pathol 2006;168:1169–78.
CONCLUSION: All solid vulvar masses should be thoroughly evaluated with a low threshold for biopsy. Treatment should consist of complete resection. Hormonal manipulation, chemotherapy, and radiation should be considered as potential adjuvant treatment options. (Obstet Gynecol 2014;124:433–5)
From the Departments of Obstetrics and Gynecology and Pathology, University of Michigan Medical Center, Ann Arbor, Michigan; and the Department of Obstetrics and Gynecology, Naval Medical Center San Diego, San Diego, California. Corresponding author: Erika L. Mowers, MD, University of Michigan Medical Center, Department of Obstetrics and Gynecology, 1500 East Medical Center Drive, L4512WH, Ann Arbor, MI 48109; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14
VOL. 124, NO. 2, PART 2, AUGUST 2014
DOI: 10.1097/AOG.0000000000000337
V
ulvar sarcomas account for 1–3% of vulvar malignancies, and leiomyosarcoma is the most common histologic variant.1,2 In addition to leiomyosarcomas, other reported vulvar sarcomas include liposarcomas, neurofibrosarcomas, rhabdomyosarcomas, dermatofibrosarcoma protuberans, angiosarcomas, Ewing sarcomas, synovial sarcomas, clear cell sarcomas, and epitheliod sarcomas.1,3,4 In a large series of 25 vulvar
Mowers et al
Bartholin Gland Myxoid Leiomyosarcoma
433
leiomyosarcomas reported by Aartsen et al,2 the mean patient age was 49.8 years, with a range from 5 to 84 years. Vulvar leiomyosarcomas are thought to originate from smooth muscle within erectile tissue, blood vessel walls, the round ligament, the dartos muscle, or the erectorpili muscle.1,3,4 Labia majora, labia minora, and pericloitoral areas are the predominant sites of occurrence. Early case series of vulvar leiomyosarcomas report a mortality rate of approximately 30-40%, but this appears to be significantly lower in more recent literature.1,2 We describe a case of a premenopausal woman with a myxoid leiomyosarcoma of the Bartholin gland. Myxoid leiomyosarcoma is an uncommon variant and is difficult to diagnose. A PubMed search was performed with the terms “vulvar leiomyosarcoma,” “Bartholin’s gland leiomyosarcoma,” and “myxoid leiomyosarcoma.” No date range was specified. Our case is rare because of both the Bartholin gland location and myxoid pathology. Only one other case of Bartholin gland myxoid leiomyosarcoma has been reported.1 The most common presentation of a vulvar leiomyosarcoma is a slow-growing, painless mass that eventually results in discomfort with intercourse or from undergarments.5 Diagnosis of these malignancies is often delayed because these lesions are mistaken for benign Bartholin gland cysts. There is a paucity of data to guide clinical management of these rare tumors, and there are varying opinions regarding the benefit of radical vulvectomy, inguinal lymphadenectomy, and bilateral salpingo-oophorectomy, as well as adjuvant chemotherapy or radiation.
CASE A 48-year-old premenopausal woman, gravida 5, para 3, initially noted a left vulvar mass in 2007. It measured approximately 335 cm and did not resolve despite oral antibiotic treatment. She underwent incision and drainage but continued to have a 3 cm area of persistent induration of the left Bartholin gland. She did not seek treatment until 5 years later, when the mass rapidly increased in size and caused pain and difficulty urinating. It was 10 cm in diameter when she underwent partial excision and marsupialization. The mass was firm and filled with blood and soft tissue. Pathology showed high-grade leiomyosarcoma with positive margins. The excisional biopsy revealed fragments of a spindle-cell neoplasm in a myxoid background. Atypical mitoses were easily identified. Immunohistochemical staining was positive with antibodies directed against smooth muscle actin, desmin, estrogen, and progesterone receptors. These findings are consistent with a diagnosis of myxoid leiomyosarcoma. Computed tomography of the chest and magnetic resonance imaging of the abdomen and pelvis were
434
Mowers et al
negative for metastatic disease, but there were masses within the uterus consistent with leiomyomas. She underwent a left radical hemivulvectomy with resection of the left Bartholin gland. Surgical margins were 2.5 cm. Final pathology revealed a myxoid leiomyosarcoma measuring 4.632.532.7 cm with positive margins. Residual myxoid leiomyosarcoma was morphologically similar to the original excision. Foreign body–type giant cells were present and associated with resorbing suture material. The tumor extended out from the identifiable Bartholin gland. Because of evidence of uterine leiomyomas on imaging, it was difficult to rule out primary uterine leiomyosarcoma with metastasis to the vulva. She therefore underwent laparoscopic hysterectomy with bilateral salpingooophorectomy as well as re-excision at the site of positive margins. Pathology showed no evidence of residual sarcoma. There were benign leiomyomas within the uterus and a benign endocervical polyp. She completed six cycles of adjuvant chemotherapy with doxorubicin and ifosfamide. She is currently 1 year from completion of adjuvant chemotherapy and 18 months from the time of diagnosis without evidence of recurrent disease.
COMMENT Nielsen et al6 proposed an expanded criteria in 1996 to distinguish leiomyosarcomas and leiomyomas of the vulva. Leiomyosarcomas are defined as meeting three or more of the following criteria: diameter more than 5 cm; infiltrative margin; mitotic count of 5 or more per 10 high-power fields; and grade 2 or 3 nuclear atypia. These tumors are rare and treatment options are varied because aggressive cases as well as late local recurrences and metastasis have been observed.5 Our patient met all criteria except for the mitotic count, which was four mitosis per 10 highpower fields. This is consistent with the diagnosis of myxoid leiomyosarcoma because these tumors often have a deceptively low mitotic count. It remains unclear if this is attributable to hypocellularity or to the fact that myxoid leiomyosarcomas tend to be better differentiated. In 2009, Gonzalez-Bugatto et al3 performed a comprehensive review of the seven reported cases of vulvar leiomyosarcomas specific to the Bartholin gland. During the follow-up of 13 months to 24 years, no cases of distant metastases were identified. Leiomyosarcomas originating in the Bartholin gland therefore may be less aggressive, with a reduced risk of hematogenous spread and distant metastasis compared with other vulvar leiomyosarcomas.3 Many benign vulvar tumors display myxoid stroma and leiomyosarcomas with myxoid changes often present a diagnostic challenge. Myxoid leiomyosarcoma is defined by Rubin et al7 as exhibiting
Bartholin Gland Myxoid Leiomyosarcoma
OBSTETRICS & GYNECOLOGY
microscopic and immunohistochemical evidence of smooth muscle differentiation and containing myxoid stroma in at least 50% of the tumor. Light microscopy generally shows intersecting fascicles of spindle cells with abundant eosinophilic cytoplasm, centrally located blunt-ended nuclei, and at least one mitosis per 10 high-power field. In the 18 cases of soft tissue myxoid leiomyosarcoma reviewed by Rubin et al,7 immunohistochemical stains were positive for smooth muscle actin (94%) and desmin (44%). Our specimen was positive for both of these markers. Myxoid variant leiomyosarcomas may be diagnosed more frequently in pregnancy because of transformation of the stroma with focal pooling of mucin.1 The clinical implications of myxoid variant leiomyosarcomas remain unclear because of the paucity of case reports. Our case describes a 3 cm area thought to have induration and subsequent scar formation that remained quiescent for approximately 5 years followed by rapid growth. Two cases of similar clinical presentations were described by Nielsen et al,6 and pathology suggested a malignant transformation in at least one of these instances. Wide local excision is the recommended treatment for vulvar leiomyosarcomas with at least a 2 cm margin. These tumors lack a real capsule and the risk of local recurrence increases significantly with inadequate margins. Inguinal lymphadenectomy is not warranted unless lymphadenopathy is present clinically.2 Adjuvant chemotherapy or radiation for completely resected vulvar leiomyosarcomas has not been shown to increase survival, although it is difficult to draw conclusions from such limited data.4 Radiation plays a role in local control and has been shown to reduce the overall burden of disease. Chemotherapy has been shown to improve survival and quality of life in patients with leiomyosarcoma of other soft tissue.5 The National Comprehensive Cancer Network recommends treating stage II or stage III uterine sarcomas with adjuvant chemotherapy (category 2A evidence). We chose six cycles of doxorubicin and ifosfamide chemotherapy in our patient given the increased risk with the large size, grade 2 histology, and positive
VOL. 124, NO. 2, PART 2, AUGUST 2014
margins after initial resection. Grade of tumor has been shown to be the most important predictor of overall outcome and local recurrence in vulvar sarcomas.5 Staining was positive for both estrogen and progesterone receptors in our case. Nielsen et al6 reported the presence of estrogen and progesterone receptors in three out of four vulvar leiomyosarcomas. Rapid growth during pregnancy, especially in the third trimester, has also been reported.1 This suggests that bilateral salpingo-oophorectomy as well as aromatase inhibitors or selective estrogen receptor modulators may have a role in treatment. Our case demonstrates a vulvar myxoid leiomyosarcoma in the Bartholin gland. Vulvar masses should be considered at increased risk for malignancy in cases of firm or solid lesions, especially those with ulceration or a rapid rate of growth. To guide treatment, a biopsy should be obtained as soon as there is any concern for malignancy. According to the current literature, treatment should consist of complete resection whenever possible. Potential adjuvant treatment options including chemotherapy, radiation, and hormonal manipulation should be considered on a case-by-case basis. REFERENCES 1. Di Gilio AR, Cormio G, Resta L, Carriero C, Loizzi V, Parisi AM, et al. Rapid growth of myxoid leiomyosarcoma of the vulva during pregnancy: a case report. Int J Gynecol Cancer 2004;14:172–5. 2. Aartsen EJ, Albus-Lutter CE. Vulvar sarcoma: clinical implications. Eur J Obstet Gynecol Reprod Biol 1994;56:181–9. 3. Gonzalez-Bugatto F, Anon-Requena MJ, Lopez-Guerrero MA, Baez-Perea JM, Bartha JL, Hervias-Vivancos B. Vulvar leiomyosarcoma in Bartholin’s gland area: a case report and literature review. Arch Gynecol Obstet 2009;279:171–4. 4. Dewdney S, Kennedy CM, Galask RP. Leiomyosarcoma of the vulva: a case report. J Reprod Med 2005;50:630–2. 5. Behranwala KA, Latifaj B, Blake P, Barton DP, Shepherd JH, Thomas JM. Vulvar soft tissue tumors. Int J Gynecol Cancer 2004;14:94–9. 6. Nielsen GP, Rosenberg AE, Koerner FC, Young RH, Scully RE. Smooth-muscle tumors of the vulva. A clinicopathological study of 25 cases and review of the literature. Am J Surg Pathol 1996; 20:779–93. 7. Rubin BP, Fletcher CDM. Myxoid leiomyosarcoma of soft tissue, an underrecognized variant. Am J Surg Pathol 2000;24: 927–36.
Mowers et al
Bartholin Gland Myxoid Leiomyosarcoma
435
ferent from these liver disorders in other settings. Influenza-mediated hepatitis would be expected to resolve spontaneously with supportive and symptomatic care over 10 to 14 days with favorable maternal and perinatal outcomes.
Myxoid Leiomyosarcoma of the Bartholin Gland
CASE: Our patient presented with a 10 cm mass on her vulva, which was presumed to be a Bartholin gland cyst. Pathology showed a high-grade myxoid leiomyosarcoma with positive margins. She was treated with left radical hemivulvectomy, laparoscopic hysterectomy with bilateral salpingo-oophorectomy, and six cycles of adjuvant chemotherapy. She is now 1 year from completion of therapy and remains disease-free.
Erika L. Mowers, MD, Jessica J. Shank, MD, Nora Frisch, MD, and R. Kevin Reynolds,
MD, FACS
BACKGROUND: Myxoid leiomyosarcomas originating from the Bartholin gland are exceedingly rare. Only one other case of a Bartholin gland myxoid leiomyosarcoma has been reported.
REFERENCES 1. Creasy RK, Resnik R, Iams JD, Lockwood CJ, Moore TR. Creasy & Resnik’s maternal-fetal medicine: principles and practice. 6th ed. Philadelphia (PA): Saunders Elsevier; 2009. 2. Lee NM, Brady CW. Liver disease in pregnancy. World J Gastroenterol 2009;15:897–906. 3. Adams DH, Hubscher SG. Commentary: systemic viral infections and collateral damage in the liver. Am J Pathol 2006;168: 1057–9. 4. Basir N, Yew TG, Telisinghe PU, Chong VH. Autoimmune hepatitis in children: a report of two cases. Singapore Med J 2011;52:e27–30. 5. Papic N, Pangercic A, Vargovic M, Barsic B, Vince A, Kuzman I. Liver involvement during influenza infection: perspective on the 2009 influenza pandemic. Influenza Other Respir Viruses 2012;6:e2–5. 6. Polakos NK, Cornejo JC, Murray DA, Write KO, Treanor JJ, Crispe IN. Kupffer cell-dependent hepatitis occurs during influenza infection. Am J Pathol 2006;168:1169–78.
CONCLUSION: All solid vulvar masses should be thoroughly evaluated with a low threshold for biopsy. Treatment should consist of complete resection. Hormonal manipulation, chemotherapy, and radiation should be considered as potential adjuvant treatment options. (Obstet Gynecol 2014;124:433–5)
From the Departments of Obstetrics and Gynecology and Pathology, University of Michigan Medical Center, Ann Arbor, Michigan; and the Department of Obstetrics and Gynecology, Naval Medical Center San Diego, San Diego, California. Corresponding author: Erika L. Mowers, MD, University of Michigan Medical Center, Department of Obstetrics and Gynecology, 1500 East Medical Center Drive, L4512WH, Ann Arbor, MI 48109; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14
VOL. 124, NO. 2, PART 2, AUGUST 2014
DOI: 10.1097/AOG.0000000000000337
V
ulvar sarcomas account for 1–3% of vulvar malignancies, and leiomyosarcoma is the most common histologic variant.1,2 In addition to leiomyosarcomas, other reported vulvar sarcomas include liposarcomas, neurofibrosarcomas, rhabdomyosarcomas, dermatofibrosarcoma protuberans, angiosarcomas, Ewing sarcomas, synovial sarcomas, clear cell sarcomas, and epitheliod sarcomas.1,3,4 In a large series of 25 vulvar
Mowers et al
Bartholin Gland Myxoid Leiomyosarcoma
433
leiomyosarcomas reported by Aartsen et al,2 the mean patient age was 49.8 years, with a range from 5 to 84 years. Vulvar leiomyosarcomas are thought to originate from smooth muscle within erectile tissue, blood vessel walls, the round ligament, the dartos muscle, or the erectorpili muscle.1,3,4 Labia majora, labia minora, and pericloitoral areas are the predominant sites of occurrence. Early case series of vulvar leiomyosarcomas report a mortality rate of approximately 30-40%, but this appears to be significantly lower in more recent literature.1,2 We describe a case of a premenopausal woman with a myxoid leiomyosarcoma of the Bartholin gland. Myxoid leiomyosarcoma is an uncommon variant and is difficult to diagnose. A PubMed search was performed with the terms “vulvar leiomyosarcoma,” “Bartholin’s gland leiomyosarcoma,” and “myxoid leiomyosarcoma.” No date range was specified. Our case is rare because of both the Bartholin gland location and myxoid pathology. Only one other case of Bartholin gland myxoid leiomyosarcoma has been reported.1 The most common presentation of a vulvar leiomyosarcoma is a slow-growing, painless mass that eventually results in discomfort with intercourse or from undergarments.5 Diagnosis of these malignancies is often delayed because these lesions are mistaken for benign Bartholin gland cysts. There is a paucity of data to guide clinical management of these rare tumors, and there are varying opinions regarding the benefit of radical vulvectomy, inguinal lymphadenectomy, and bilateral salpingo-oophorectomy, as well as adjuvant chemotherapy or radiation.
CASE A 48-year-old premenopausal woman, gravida 5, para 3, initially noted a left vulvar mass in 2007. It measured approximately 335 cm and did not resolve despite oral antibiotic treatment. She underwent incision and drainage but continued to have a 3 cm area of persistent induration of the left Bartholin gland. She did not seek treatment until 5 years later, when the mass rapidly increased in size and caused pain and difficulty urinating. It was 10 cm in diameter when she underwent partial excision and marsupialization. The mass was firm and filled with blood and soft tissue. Pathology showed high-grade leiomyosarcoma with positive margins. The excisional biopsy revealed fragments of a spindle-cell neoplasm in a myxoid background. Atypical mitoses were easily identified. Immunohistochemical staining was positive with antibodies directed against smooth muscle actin, desmin, estrogen, and progesterone receptors. These findings are consistent with a diagnosis of myxoid leiomyosarcoma. Computed tomography of the chest and magnetic resonance imaging of the abdomen and pelvis were
434
Mowers et al
negative for metastatic disease, but there were masses within the uterus consistent with leiomyomas. She underwent a left radical hemivulvectomy with resection of the left Bartholin gland. Surgical margins were 2.5 cm. Final pathology revealed a myxoid leiomyosarcoma measuring 4.632.532.7 cm with positive margins. Residual myxoid leiomyosarcoma was morphologically similar to the original excision. Foreign body–type giant cells were present and associated with resorbing suture material. The tumor extended out from the identifiable Bartholin gland. Because of evidence of uterine leiomyomas on imaging, it was difficult to rule out primary uterine leiomyosarcoma with metastasis to the vulva. She therefore underwent laparoscopic hysterectomy with bilateral salpingooophorectomy as well as re-excision at the site of positive margins. Pathology showed no evidence of residual sarcoma. There were benign leiomyomas within the uterus and a benign endocervical polyp. She completed six cycles of adjuvant chemotherapy with doxorubicin and ifosfamide. She is currently 1 year from completion of adjuvant chemotherapy and 18 months from the time of diagnosis without evidence of recurrent disease.
COMMENT Nielsen et al6 proposed an expanded criteria in 1996 to distinguish leiomyosarcomas and leiomyomas of the vulva. Leiomyosarcomas are defined as meeting three or more of the following criteria: diameter more than 5 cm; infiltrative margin; mitotic count of 5 or more per 10 high-power fields; and grade 2 or 3 nuclear atypia. These tumors are rare and treatment options are varied because aggressive cases as well as late local recurrences and metastasis have been observed.5 Our patient met all criteria except for the mitotic count, which was four mitosis per 10 highpower fields. This is consistent with the diagnosis of myxoid leiomyosarcoma because these tumors often have a deceptively low mitotic count. It remains unclear if this is attributable to hypocellularity or to the fact that myxoid leiomyosarcomas tend to be better differentiated. In 2009, Gonzalez-Bugatto et al3 performed a comprehensive review of the seven reported cases of vulvar leiomyosarcomas specific to the Bartholin gland. During the follow-up of 13 months to 24 years, no cases of distant metastases were identified. Leiomyosarcomas originating in the Bartholin gland therefore may be less aggressive, with a reduced risk of hematogenous spread and distant metastasis compared with other vulvar leiomyosarcomas.3 Many benign vulvar tumors display myxoid stroma and leiomyosarcomas with myxoid changes often present a diagnostic challenge. Myxoid leiomyosarcoma is defined by Rubin et al7 as exhibiting
Bartholin Gland Myxoid Leiomyosarcoma
OBSTETRICS & GYNECOLOGY
microscopic and immunohistochemical evidence of smooth muscle differentiation and containing myxoid stroma in at least 50% of the tumor. Light microscopy generally shows intersecting fascicles of spindle cells with abundant eosinophilic cytoplasm, centrally located blunt-ended nuclei, and at least one mitosis per 10 high-power field. In the 18 cases of soft tissue myxoid leiomyosarcoma reviewed by Rubin et al,7 immunohistochemical stains were positive for smooth muscle actin (94%) and desmin (44%). Our specimen was positive for both of these markers. Myxoid variant leiomyosarcomas may be diagnosed more frequently in pregnancy because of transformation of the stroma with focal pooling of mucin.1 The clinical implications of myxoid variant leiomyosarcomas remain unclear because of the paucity of case reports. Our case describes a 3 cm area thought to have induration and subsequent scar formation that remained quiescent for approximately 5 years followed by rapid growth. Two cases of similar clinical presentations were described by Nielsen et al,6 and pathology suggested a malignant transformation in at least one of these instances. Wide local excision is the recommended treatment for vulvar leiomyosarcomas with at least a 2 cm margin. These tumors lack a real capsule and the risk of local recurrence increases significantly with inadequate margins. Inguinal lymphadenectomy is not warranted unless lymphadenopathy is present clinically.2 Adjuvant chemotherapy or radiation for completely resected vulvar leiomyosarcomas has not been shown to increase survival, although it is difficult to draw conclusions from such limited data.4 Radiation plays a role in local control and has been shown to reduce the overall burden of disease. Chemotherapy has been shown to improve survival and quality of life in patients with leiomyosarcoma of other soft tissue.5 The National Comprehensive Cancer Network recommends treating stage II or stage III uterine sarcomas with adjuvant chemotherapy (category 2A evidence). We chose six cycles of doxorubicin and ifosfamide chemotherapy in our patient given the increased risk with the large size, grade 2 histology, and positive
VOL. 124, NO. 2, PART 2, AUGUST 2014
margins after initial resection. Grade of tumor has been shown to be the most important predictor of overall outcome and local recurrence in vulvar sarcomas.5 Staining was positive for both estrogen and progesterone receptors in our case. Nielsen et al6 reported the presence of estrogen and progesterone receptors in three out of four vulvar leiomyosarcomas. Rapid growth during pregnancy, especially in the third trimester, has also been reported.1 This suggests that bilateral salpingo-oophorectomy as well as aromatase inhibitors or selective estrogen receptor modulators may have a role in treatment. Our case demonstrates a vulvar myxoid leiomyosarcoma in the Bartholin gland. Vulvar masses should be considered at increased risk for malignancy in cases of firm or solid lesions, especially those with ulceration or a rapid rate of growth. To guide treatment, a biopsy should be obtained as soon as there is any concern for malignancy. According to the current literature, treatment should consist of complete resection whenever possible. Potential adjuvant treatment options including chemotherapy, radiation, and hormonal manipulation should be considered on a case-by-case basis. REFERENCES 1. Di Gilio AR, Cormio G, Resta L, Carriero C, Loizzi V, Parisi AM, et al. Rapid growth of myxoid leiomyosarcoma of the vulva during pregnancy: a case report. Int J Gynecol Cancer 2004;14:172–5. 2. Aartsen EJ, Albus-Lutter CE. Vulvar sarcoma: clinical implications. Eur J Obstet Gynecol Reprod Biol 1994;56:181–9. 3. Gonzalez-Bugatto F, Anon-Requena MJ, Lopez-Guerrero MA, Baez-Perea JM, Bartha JL, Hervias-Vivancos B. Vulvar leiomyosarcoma in Bartholin’s gland area: a case report and literature review. Arch Gynecol Obstet 2009;279:171–4. 4. Dewdney S, Kennedy CM, Galask RP. Leiomyosarcoma of the vulva: a case report. J Reprod Med 2005;50:630–2. 5. Behranwala KA, Latifaj B, Blake P, Barton DP, Shepherd JH, Thomas JM. Vulvar soft tissue tumors. Int J Gynecol Cancer 2004;14:94–9. 6. Nielsen GP, Rosenberg AE, Koerner FC, Young RH, Scully RE. Smooth-muscle tumors of the vulva. A clinicopathological study of 25 cases and review of the literature. Am J Surg Pathol 1996; 20:779–93. 7. Rubin BP, Fletcher CDM. Myxoid leiomyosarcoma of soft tissue, an underrecognized variant. Am J Surg Pathol 2000;24: 927–36.
Mowers et al
Bartholin Gland Myxoid Leiomyosarcoma
435
