Baylor University Medical Center Proceedings January 2014, Volume 27, Number 1 | Page 29

duct cyst can result in a delay of diagnosis and worse prognosis
(7).
Both leiomyomas and leiomyosarcomas are immunopositive
for muscle markers, including desmin, smooth muscle actin, and
muscle-specific actin, and they can be focally positive for S-100
and cytokeratin. The diagnosis of extrauterine leiomyosarcomas
in the gynecologic tract requires the presence of at least three
of the following characteristics: a diameter >5 cm, infiltrative
margins, >5 mitotic figures per 10 HPF, and moderate to severe
cytologic atypia. Lesions that have only one of these characteristics should be diagnosed as leiomyomas, and cases with two
characteristics should be considered atypical leiomyomas (8).
Due to the low incidence of these tumors, there are no evidence-based diagnostic algorithms or published recommendations for treatment. However, prior reports have recommended
surgical excision with the potential addition of radiation therapy.
Decisions are made based upon the individual case presentation and pathology evaluation. Leiomyosarcomas are generally
treated by complete excision with a goal of pathologic confirmation of negative margins. Conversations between pathologists
and clinicians can provide guidance to ensure adequate surgical
excisions are performed. Prior studies have shown that risk of
recurrence is most closely related to inadequate resection of
margins (9). The overall prognosis is best correlated to histologic
grade (5). Close monitoring of the patient is advised, as these
entities have almost a 50% recurrence rate (5).
The value of adjuvant chemotherapy is uncertain but has
produced regression of metastases in vulvar sarcomas (9).

January 2014

Adjuvant chemotherapy and radiation therapy for completely
resected low-grade mesenchymal tumors have not been shown
to improve outcomes (3, 5). Small case series have shown benefit
in treating high-grade sarcomas or recurrent low-grade sarcomas with postoperative radiation; however, it is very difficult
to compare treatment regimens at different institutions as there
are no standardized guidelines (5).
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Misumi S, Irie T, Fukuda K, Tada S, Hosomura Y. A case of deep soft
tissue leiomyoma: CT and MRI findings. Radiat Med 2000;18(4):253–
256.
Kumar V. The female genital tract. In Kumar V, Abbas AK, Fausto N, Aster
J, eds. Robbins and Cotran Pathologic Basis of Disease, 8th ed. Philadelphia:
Saunders Elsevier, 2010:1005–1062.
DiSaia PJ, Pecorelli S. Gynecological sarcomas. Semin Surg Oncol
1994;10(5):369–373.
Lösch A, Joura EA, Stani J, Breitenecker G, Lahodny J. Leiomyosarcoma
of the vulva. A case report. J Reprod Med 2001;46(6):609–612.
Curtin JP, Saigo P, Slucher B, Venkatraman ES, Mychalczak B, Hoskins
WJ. Soft-tissue sarcoma of the vagina and vulva: a clinicopathologic study.
Obstet Gynecol 1995;86(2):269–272.
Dewdney S, Kennedy CM, Galask RP. Leiomyosarcoma of the vulva: a
case report. J Reprod Med 2005;50(8):630–632.
González-Bugatto F, Añón-Requena MJ, López-Guerrero MA, BáezPerea JM, Bartha JL, Hervías-Vivancos B. Vulvar leiomyosarcoma in
Bartholin’s gland area: a case report and literature review. Arch Gynecol
Obstet 2009;279(2):171–174.
Nielsen GP, Rosenberg AE, Koerner FC, Young RH, Scully RE. Smoothmuscle tumors of the vulva. A clinicopathological study of 25 cases and
review of the literature. Am J Surg Pathol 1996;20(7):779–793.
Aartsen EJ, Albus-Lutter CE. Vulvar sarcoma: clinical implications. Eur
J Obstet Gynecol Reprod Biol 1994;56(3):181–189.

Smooth muscle neoplasms of the vulva masquerading as Bartholin gland duct cysts

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