that measured 4 ⫻ 3 cm. Microscopically, the neoplasm was a nerve sheath tumor that showed nodules of pale epithelial cells separated by fascicles of spindle cells with myxomatous stroma. There was no evidence of mitotic activity (Figure 1). Immunoperoxidase stains for smooth muscle actin were negative but vimentin positive, which excludes smooth muscle tumors and confirmed nerve sheath origin. The diagnosis was a benign schwannoma. This woman was seen 6 weeks later in the gynecology outpatient clinic. The wound had healed satisfactorily and she was asymptomatic. The benign nature of the tumor was discussed and the patient reassured. She was reported to be asymptomatic 6 months postoperatively. COMMENT In conclusion, a schwannoma is a rare benign tumor that arises in deeper tissues from a nerve sheath. These tumors are extremely difficult or even impossible to
Tubulovillous Adenoma of the Vulva D. Vitrey, MD, S. Frachon, MD, B. Balme, MD, and F. Golfier, MD, PhD Departments of Pathology, Gynecology, and Dermatopathology, Hoˆtel Dieu, Lyon, France
BACKGROUND: We report the second case of enteric-type villous adenoma of the vulva. The differential diagnosis, histogenesis, and pathogenesis are discussed. CASE: A 66-year-old woman had a tumor resected from the posterior aspect of the vulva. The tumor was characterized by its villous architecture, with columnar epithelium and goblet cells. This vulvar tubulovillous adenoma was identical to a tumor resected 6 months before from this patient’s rectal wall. CONCLUSION: These two tumors behaved benignly, and the vulvar adenoma possibly originated with the mu¨llerian vestige. (Obstet Gynecol 2003;102:1160 –3. © 2003 by The American College of Obstetricians and Gynecologists.)
Tubulovillous adenomas are common in the colon and rectum, where they can turn malignant. The vulvar Address reprint requests to: Danie`le Vitrey, Department of Pathology, Hoˆtel Dieu, 1 place de l’Hoˆpital, 69288 LYON Cedex 02, France; E-mail:
[email protected].
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diagnose preoperatively. The initial thinking in this case was that the tumor was an ovarian mass. If clinicians are faced with an inguinal swelling or herniation and a coexisting abdominal mass, they should perhaps consider other radiologic studies that might better identify the origin. REFERENCES 1. Fletcher CDM. Peripheral nerve sheath tumors. A clinicopathological update. Pathol Annu 1990;25:53–74. 2. White W, Shiu MH, Rosenblum MK, Erlandson RA, Woodruff JM. Cellular schwannoma. A clinicopathologic study of 57 patients and 58 tumors. Cancer 1990;66:1266–75. 3. Karakousis CP. Abdominoinguinal incision and other incisions in the resection of pelvic tumors. Surg Oncol 2000;9: 83–90. Received December 11, 2002. Received in revised form February 3, 2003. Accepted February 27, 2003.
location of this tumor is very rare, as only one case has been reported in the literature.3 Some of these tumors have been reported in the vagina, the uterine cervix, and the paraurethral region.1–3,8 Colonic-type adenocarcinomas have been reported in this vulvar location, with persistence of villous adenoma patterns.4 –7 We here report a second case of this rare association of vulvar and rectal tubulovillous adenomas.
CASE A 66-year-old woman was treated 6 months previously for a rectal villous adenoma by transanal surgical resection. This oval tumor measured 6.5 ⫻ 5 cm and had developed in the left lateral wall of the rectum, 7 cm above the anus. Histologically, this tumor displayed the typical aspect of villous adenomas without any sign of malignancy. Six months later, vulvar pruritus led to the discovery of an exophytic and erythematous tumor measuring 2 cm in diameter, with a smooth surface. It was located at the posterolateral aspect of the vestibule (Figure 1). Subsequently, the patient was subjected to anoscopy examination, colonoscopy, and echoendoscopy. All of these investigations yielded negative results. A rectal biopsy showed no recurrence of the rectal adenoma. The vulvar tumor biopsy was not a contributory factor in the diagnosis leading to a radical surgical procedure. A resection of the vulvar scar was performed 2 weeks later, as surgical margins on the initial resection were uncertain.
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Figure 1. Gross appearance of lesion: erythematous exophytic tumor of posterolateral left side of vestibule. Vitrey. Tubulovillous Adenoma. Obstet Gynecol 2003.
No residual adenoma was found. One year later clinical examination was normal. Histopathologic studies of the rectal tumor, vulvar biopsy, and vulvar tumor were done. The sections were stained with the hematoxylin– eosin–saffron method. The examination of the vulvar tumor was completed with periodic acid–Schiff staining with and without amylase digestion and with alcian blue at pH 2.5 by standard techniques, to look for digestive-like mucin. Additional sections were obtained from the paraffin blocks for immunohistochemical studies. The standard avidin– biotin
peroxidase technique was used with polyclonal carcinoembryonic antigen (dilution 1:100) (Dako, Glostrup, Denmark), Ki67 antigen (dilution 1:30) (Immunotech, Marseilles, France), and p53 antigen (dilution 1:25) (Dako). Control standard tests were performed. Gross examination of the surgical specimen revealed a vulvar tumor measuring 3.5 ⫻ 3.5 cm, with an exophytic lesion of 1.8 ⫻ 1.2 cm. The cutting section displayed a papillary architectural pattern, without stromal invasion. The tumor seamed to involve the anterior and right lateral margins.
Figure 2. Microscopic pattern: villous glandular architecture of the vulvar tumor evoking a villous rectal tumor. ⫻ 25, original magnification. Vitrey. Tubulovillous Adenoma. Obstet Gynecol 2003.
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Figure 3. Villous morphology: minimal dysplastic mucinous columnar cell without atypic cells. Hematoxylin– eosin–saffron staining; ⫻ 200, original magnification. Vitrey. Tubulovillous Adenoma. Obstet Gynecol 2003.
Microscopic examination of the tumor revealed a villous architectural lesion lined by a superficial glandular proliferation arising in continuity with adjacent normal squamous epithelium (Figure 2). The lesion was composed of fine papillary fronds lined by a pseudostratified layer of columnar epithelial cells with some mucinous cells without cellular atypia (Figure 3). Rare mitoses were observed. Staining sections confirmed the presence of intracytoplasmic mucin production (periodic acid–Schiff positive with and without amylase digestion and alcian blue staining at pH 2.5). Strong and diffuse immunoreactivity to carcinoembryonic antigen was observed in epithelial tumor cells of adenoma. Ki67 and p53 stains were negative. The transanal rectal surgical specimen measured 6.5 ⫻ 5 cm and was entirely examined. Histological features of the tumor were a villous frond proliferation and tubular pattern lined by a layer of cylindric cells, with mild and moderate dysplasic cells occupying two thirds
of the villous epithelium. There was an area of 5 mm in diameter with severe dysplasia. The basement membrane was intact, and the stroma underneath showed a moderately chronic inflammation and fibrosis. We compared the two tumors. The architectural pattern was similar and no malignant cells were present in these two localizations.
COMMENT Exophytic vulvar tumors usually develop from squamous epithelium or from adnexal glands. Vulvar papillomas are very common nonkeratinized stratified squamous benign tumors. Glandular tumors of the vulva are less frequent and mainly represented by cystic papillary hidradenoma, syringoma, and cystic formations developed from the adnexal glands. Ectopic tissues such as breast ectopies, mu¨llerian tissues, and remains of mesonephric origin can also be responsible for exophytic
Table 1. Reports of Enteric-Type Tumors of the Female Genital Tract Study
Age (y)
Site 1
Site 2
Histology
72 35 43 59 54 63 80 57
Cervix Rectum (1967) Vagina Cervix Vulva Vulva Vulva Vulva Vulva
0 Vagina (1986) 0 0 0 0 0 0 0
Enteric-type adenocarcinoma Tubulovillous adenomas Enteric-type adenocarcinoma Enteric-type adenocarcinoma Tubulovillous adenoma Enteric-type microinvasive adenocarcinoma Enteric-type microinvasive adenocarcinoma Enteric-type adenocarcinoma Enteric-type adenocarcinoma
1
Michael Ciano2 Fox3
Kennedy4 Ghamande5 Willen6
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lesions of the vulva. Reports of ectopic vulvar tumors such as tubulovillous adenomas are very rare. A review of the literature showed the uncommon characteristics of our observation, as only one case of vulvar adenoma has been reported among several cases of vulvar, vaginal, or cervical enteric-type adenocarcinomas (Table 1). The case of vulvar villous adenoma was reported by Fox3 in a 59-year-old woman without associated rectal lesion. Ciano2 reported an association of enteric-type villous adenoma of the lower third of the vagina in a 72-year-old woman who 18 years previously had surgical resection of a villous adenoma of the rectum. Four cases of adenocarcinomas were reported in the vulva,4,5,7 two on the cervix,1,3 and one in the vagina.2 Histologically, these lesions were well-differentiated enteric-type adenocarcinomas. These tumors were developed in continuity with the squamous epithelium. Numerous theories have been proposed in the literature regarding the histogenesis and pathogenesis of these lesions.3,4,6 In our case, we can rule out the hypothesis of intestinal metaplasia of the adnexal vulvar glands (sudoriferous and sebaceous), the periurethral or Skene glands, and the minor and major vestibular glands (Bartholin glands). Actually, the posterior location is incompatible with a periurethral origin, and the proliferating growth pattern and the lack of continuity with the gland allow us to eliminate an origin from the minor vestibular glands. The vagina is developed from the cloaca, around the seventh week of gestation. Division of the cloaca occurs with separation of the anterior urogenital sinus from the posterior anorectal canal by an intervening urorectal septum. Later, the urogenital sinus is divided into the bladder, the pelvic urethra, and the vestibule of the
vagina. Changes in the septum result in entoblastic tissue proliferation and then the presence of intestinal mucosal epithelium. Therefore, we agree with Willen et al and Kennedy et al,4,6 who suggested that the presence of heterotopic glandular tissue could be the origin of vulvar villous adenomas.
Use of a Vacuum-Assisted Closure Device in the Treatment of Recurrent Paget’s Disease of the Vulva
BACKGROUND: Paget’s disease of the vulva is a rare malignancy for which common recurrence is related to inadequate surgical margins. Excision with adequate surgical margins might leave a large defect that does not readily heal.
Hyun S. Shvartsman, MD, Howard Langstein, MD, Cynthia Worley, RN, Anais Malpica, MD, and Lois M. Ramondetta, MD Departments of Gynecologic Oncology, Plastic Surgery, Nursing–Wound Ostomy and Continence, and Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas Address reprint requests to: Hyun S. Shvartsman, MD, The University of Texas MD Anderson Cancer Center, Department of Gynecologic Oncology, 1515 Holcombe Boulevard, Unit 440, Houston, TX 77030-4009; E-mail:
[email protected].
REFERENCES 1. Michael H, Sutton G, Hull MT. Villous adenoma of. the uterine cervix associated with invasive adenocarcinoma. Int J Gynecol Pathol 1986;5:163. 2. Ciano PS, Antonioli DA, Critchlow J, Burke L, Goldman H. Villous adenoma presenting as a vaginal polyp in a rectovaginal tract. Hum Pathol 1987;18:863–6. 3. Fox H, Wells M, Harris M, McWilliam LJ, Anderson GS. Enteric tumors of the lower femal genital tract: A report of three cases. Histopathology 1988;12:167–76. 4. Kennedy JC, Majmudar B. Primary adenocarcinoma of the vulva, possibly cloagenic. A report of two cases. J Reprod Med 1993;38:113–6. 5. Ghamande SA, Kaszinca J, Griffiths CT, Finkler NJ, Hamid AM. Mucinous adenocarcinomas of the vulva. Gynecol Oncol 1995;57:117–20. 6. Willen R, Bedassy Z, Carlen B, Bozoky B, Cajander S. Cloacogenic adenocarcinoma of the vulva. Gynecol Oncol 1999;74:298–301. 7. Tiltman A, Knutzen VK. Primary adenocarcinoma of the vulva originating in misplaced cloacal tissue. Obstet Gynecol 1978;51:30–3. 8. Trotter SE, Philip B, Luck R, Ali M, Fisher C. Villous adenoma of the bladder. Histopathology 1994;24:491–3. Received March 4, 2002. Received in revised form October 31, 2002. Accepted December 18, 2002.
CASE: A 41-year-old woman with recurrent Paget’s disease of the vulva underwent a skinning excision of the vulva and perineum. A vacuum-assisted closure device was placed on the wound until final pathologic analysis confirmed the adequacy of surgical margins. CONCLUSION: The vacuum-assisted closure device not only allowed us to safely temporize wound closure until the final histopathologic results, but also helped secure the skin graft to the large and irregular surface. Additional studies are needed to determine the effectiveness of vacuum-assisted closure device therapy in Paget’s disease of the vulva. (Obstet Gynecol 2003;102:1163– 6. © 2003 by The American College of Obstetricians and Gynecologists.)
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