Download Report

562 Gynaecology case reports
References
Donnez J., Donnez O., Squif et J. and Nisolle M. (2001) The concept of retroperitoneal adenomyotic disease is born, in: An atlas of operative laparoscopy and
hysteroscopy, 2nd edn, edited by Donnez J. and Nisolle M., Chapter 12, pp.
113–117. London, Parthenon.
Enatsu A., Harada T., Yoshida S., Iwabe T and Terakawa N. (2000) Adenomyosi s
in a patient with the Rokitansky–Kuster–Hauser syndrome. Fertility and
Sterility, 73, 862–683.
Mitchell C.S., Goske M.J. and Applegate K. (1999) Imaging of Mullerian anomalies, in: Congenital malformations of the female genital tract—diagnosis and
management , edited by Gidwani G. and Falcone T., Chapter 4, pp. 55–57.
Philadelphia, Lippincott Williams and Wilkins.
Angiomyo broblastoma of the vulva occurring in a
teenaged girl
H. S. QUBLAN,1 L. M. FAYYAD2 and A. S. AL-GHOWERI1
Departments of 1Obstetrics and Gynecology and 2Department of Histopathology, King Hussein Medical
Center, Amman, Jordan
Angiomyo broblastoma (AMFB) is an angiomyxoma that is related to a
group of rare mesenchymal myxoid tumours (Ustun et al., 1998). This
recently described benign tumour most commonly involves the soft tissue of the vulva and vagina (Fletcher et al., 1992; Vav Der Griend et al.,
1994; Ockner et al., 1997). Patients are usually premenopausal and present with a vulval mass that is often diagnosed initially as a Bartholin’s
cyst. This benign tumour should be distinguished from the aggressive
angiomyxoma (AA) that has more in ltrative character and high recurrence rate (Hilgers et al., 1986). In this report, we describe a case of
angiomyo broblastoma in a 17-year-old female patient. To our knowledge, this is the rst case of angiomyo broblastoma seen in a teenager.
Case report
A 17-year-old female patient, single, presented with a painless mass of
the right vulva, which has been noticed rst by the patient 1 year previously. Menarche was at the age of 13, and cycles were regular.
Examination revealed a well-circumscribed, mobile swelling located in
the subcutaneous tissue of the right vulva. The preoperative clinical diagnosis was that of Bartholin gland cyst. The lesion was treated by simple
excision. The postoperative coarse was uncomplicated, and follow-up for
3 years after excision revealed no recurrence of the tumour.
Tumour description
Macroscopically, the specimen was composed of an intact lobulated and
encapsulated mass measuring 8 ´ 5 ´ 4 cm. Its cut surface was solid,
rm and greyish in colour. Microscopically, the haematoxylin and eosin
staining revealed an encapsulated neoplasm with a biphasic pattern of
proliferating thin-walled blood vessels of variable diameters and of
spindled and plump oval stromal cells arranged in hypocellular and
hypercellular areas (Fig. 1). The tumour cells had bland nuclei with
mitotic gures ranging from 0 to 1/10 HPF. Variable areas of intervening dense collagen bundles were noted as well as areas of oedematous
and myxoid stroma. Scattered lymphocytes and mast cells were seen
throughout the lesion. There was no evidence of nuclear atypia,
adipocytes, histiocytes or necrosis. Immunohistochemicall y, the stromal
Figure 1. Hypercellular and hypocellular areas of spindle cell proliferation with
collagen bundles (H&E stain, ´ 24).
Figure 2. Thin-walled vessels of variable diameters and positive immunstaining
for muscle-speci c actin in the spindle cells using anti-muscle-speci c actin
(HHF-35, Dakopatts) (´ 60).
cells were positive for muscle speci c actin (Fig. 2), vimentin and
desmin, but negative for S-100 protein, cytokeratin, CD34, oestrogen
and progesterone receptors. The nal diagnosis was angiomyo broblastoma of the vulva.
Discussion
Angiomyo broblastoma is a rare benign tumour that involving most
commonly the soft tissue of the vulva and vagina (Fletcher et al., 1992;
Laskin et al., 1998), but rarely it may arises from the female urethra
(Kitamura et al., 1999) and fallopian tube (Kobayashi et al., 1999). This
tumour was also reported to involve adult men, where it arises from the
scrotum or inguinal region (Laskin et al., 1998). Only about 73 cases
were reported in the literature, and the patient’s age in these reports
ranged from 23 to 86 years. The histogenesis of AMF is unknown. It
is possible that these tumours originate from primitive mesenchyma l
cells that may have potential for diverse lines of differentiation
(Kobayashi et al., 1999), as some of these tumours have been reported
to be mitotically active (Takeshima et al., 1998) to contain heterogeneous components, (Fokunaga et al., 1997) and, rarely, can undergo
sarcomatous change (Neilsen et al., 1997). The slow-growth wellcircumscribed tumour usually presents as a painless vulval mass and is
often misdiagnosed as a Bartholin’s gland cyst, hydrocele of the canal
of Nuck or aggressive angiomyxoma (Fletcher et al., 1992; HernandezMonge et al., 2000). Some reports showed that 1–25 years after simple
excision of the tumour there was no evidence of recurrence (Fletcher
et al., 1992; Laskin et al., 1997).
Vulval angiomyo broblastoma may be mistaken for myxoid epithelioid leiomyoma because of the presence of plump cells exhibiting
desmin immunoreactivit y. However, these benign tumours are more cellular, lack prominent vascularity, and usually show positive staining for
MSA. They have prominent myoid features ultrastructurally in the form
of micro laments with focal densities and attachment plaques, which are
poorly developed in AMFB (Fletcher et al., 1992). Angiomyo broblastoma should be distinguished from other soft-tissue tumours, most
importantly aggressive angiomyxoma, because of the differences in
clinical and pathological features, surgical management and follow-up.
Gynaecology case reports
Clinically, angiomyo broblastoma usually presents as a painless
vulval mass measuring 0.5–12 cm (usually < 5 cm) noticed for periods
ranging from 10 weeks to 8 years and often misdiagnosed as a
Bartholin’s gland cyst. In contrast, the aggressive angiomyxoma is seen
potentially over a wider anatomic spectrum, where it presents as a mass
of the pelvic soft tissue, retroperitoneum, urinary bladder, vagina, vulva
and perineum measuring 3–60 cm (usually > 5 cm) that is noticed for
short period, usually a few months (Fletcher, 1992). Pathologically, both
tumours contain myxoid stroma that is more uniform in aggressive
angiomyxoma and do not demonstrate areas of hypercellularity or dense
collagen deposition as in angiomyo broblastoma. Furthermore, the
blood vessels of angiomyo broblastoma are thin-walled, venular or capillary-sized, whereas those of aggressive angiomyxoma are characterised by large and thick-walled vascular channels (Okner et al., 1997).
Immunohistologically, Okner et al (1997) demonstrated strong similarities in the immunohistological and ultrasrtucural pro les of both
aggressive angiomyxoma and angiomyo broblastoma. Comparing three
cases of AA with 10 cases of AMFBs, they found that the stromal cells
in both groups showed positivity for vimentin, desmin, speci c muscle
actin (SMA) and oestrogen receptor protein. Contrary to these results,
in a previous study Fletcher et al. (1992) found that the stromal cells
of AMFB were positive for vimentin and desmin, negatively for SMA,
while those of AA were positive for vimentin and negative for both
desmin and SMA. However, immunohistochemical study in our case
showed the positivity of stromal cells for SMA, vimentin and desmin,
but was negative for oestrogen and progesterone receptor protein. These
results suggest the limitation of immunohistochemical studies in differentiation between the two entities. Unlike AMFB which, treated by
simple excision, resection of AA with wide tumour-free margins is the
treatment of choice to prevent recurrence of the tumour. In conclusion,
angiomyo broblastoma is a recently described benign soft tissue
tumour that should be distinguished from the aggressive angiomyxoma .
The differentiation between the two tumours should be based on clinical behaviour and histological studies. With this report, the patient’s age
ranges from 17 to 86 years.
563
References
Fletcher C.D., Tsang W.Y., Fisher C., Lee K.C. and Chan J.K. (1992)
Angiomyo broblastoma of the vulva: a benign neoplasm distinct from aggressive angiomyxoma. American Journal of Surgical Patholog y, 16, 373–382.
Fukunaga M., Nomura K., Matsumoto K., Doi K., Endo Y. and Ushigome S.
(1997) Vulval angiomyo broblastoma: clinicopathologic analysis of six cases.
Hernandez-Monge A., Estrada-Moscoso I., Alanis-Lopez P. and Villanueva L.A.
(2000) Vulvar angiomyo broblastoma. Report of a case and review of the literature. Ginecologia Y Obstetricia de Mexico, 68, 31–34.
Hilgers R.D., Pai R., Bartow S.A., Aisenbrey G. and Bowling M.C. (1986)
Aggressive angiomyxoma of the vulva. Obstetrics and Gynecology, 68,
605–625.
Kitamura H., Miyao N., Sato Y., Matsukawa M., Tsukamoto T. and Sato T. (1999)
angiomyo broblastoma of the female urethra. International Journal of Urology,
6, 268–270.
Kobayashi T., Suzuki K., Arai T. and Sugimura H. (1999) Angiomyo broblastoma
arising from the fallopian tube. Obstetrics and Gynecology, 94, 833–834.
Laskin W.B., Fetsch J.F. and Tavassoli F.A. (1997) Angiomyo broblastoma of the
female genital tract: analysis of 17 cases including a lipomatous variant. Human
Pathology , 28, 1046–1055.
Laskin W.B., Fetsch J.F.A and Mosto F.K. (1998) Angiomyo broblastoma like
tumor of the male genital tract: analysis of 11 cases with comparison to female
angiomyo broblastoma and spindle cell lipoma. American Journal of Surgical
Pathology , 22, 6–16.
Nielsen G.P., Young R.H., Dickersin G.R. and Rosenberg A.E. (1999)
Angiomyo broblastoma of the vulva with sarcomatous transformation.
American Journal of Surgical Pathology, 21, 1104–1108.
Ockner D.M., Sayadi H., Swanson P.E., Ritter J.H. and Wick M.R. (1997) Genital
angiomyo broblastoma: comparison with aggressive angiomyxoma and other
myxoid neoplasms of skin and soft tissue. American Journal of Clinical
Pathology , 107, 36–44.
Takeshima Y., Shinkoh Y. and Inai K. (1998) Angiomyo broblastoma of the vulva:
a mitotically active variant? Pathology International, 48, 292–296.
Ustun C., Malazgirt Z., Kandemir B., Kocak I., Bolat I. and Gumus S. (1998)
Angiomyo broblastoma of the vulva: case report. Pathology Internationa l, 48,
964–966.
Vav Der Griend M.D., Burda P. and Ferrier A.J. (1994) Case report.
Angiomyo broblastoma of the vulva. Gynecologic Oncology, 54, 389–392.
Correspondence to: Hussein S. Qublan, Irbid-Aidun, P.O. Box 97, Jordan. Fax: +962 2 7103268; E-mail: [email protected]
Haemangiopericytoma of the sigmoid mesocolon. An
unexpected nding during laparoscopic tubal
evaluation
R. GAZVANI, P. M. KING, W. D. THOMPSON, D. W. NOBLE and M. HAMILTON
Grampian University Hospitals, Foresterhill, Aberdeen, UK
Case report
A 37-year-old healthy woman (Mrs P.S) and her husband were seen in
the infertility clinic with a history of primary infertility of 18 months’
duration. Previous investigations of the couple con rmed regular ovulation and suboptimal semen quality. Abdominal examination as well
as transvaginal ultrasonography of the pelvis revealed normal ndings
and she was listed for a laparoscopic evaluation of her tubal patency
(lap and dye) to be carried out as a day case.
During laparoscopic examination a solid, vascular, red tumour measuring 6.5 ´ 5.5 ´ 5 cm was noted on the sigmoid colon. Several
enlarged vessels could be made out on its surface, measuring up to
0.5 cm in diameter. On closer examination, it appeared to be arising from
sigmoid mesocolon. It was partially mobile. During the investigation of
the mass, possibly due to disturbance caused by instruments, active
bleeding started from one of the vessels. A decision was made to proceed to laparatomy and the mass was removed. It was readily dissected
off the colon without any bowel injury and appeared to be encapsulated.
The histopathological investigation concluded that the tumour was
highly vascular, which was con rmed by CD34 immunostaining. There
was a regular pattern of uniform nuclei around these small blood vessels
and the appearances were of those of a haemangiopericytoma. The presence of a sharp margin, scarcity of mitoses and lack of pleomorphism
were suggested to be reassuring regarding a reduced malignant potential despite the presence of central necrosis (Fig. 1). No further treatment was indicated. A long-term follow-up was nevertheless advised
as these lesions could be unpredictable.
Further investigations including whole body computed tomography
(CT) also found no evidence of further lesions elsewhere. The patient
made an uneventful recovery and was discharged home 6 days after the
operation to be reviewed in the outpatient clinic.
In the management of her infertility, superovulation with intrauterine
insemination was carried out 6 months after the initial operation. The
patient conceived following one cycle of treatment and an intrauterine,
triplet pregnancy was con rmed by ultrasound scan. Pregnancy progressed uneventfully and a caesarean section was carried out at 34 weeks
gestation delivering three healthy infants. At the time of section, there
was no evidence of recurrence of the tumour on the pelvic colon.
Discussion
Haemangiopericytoma is a rare and solitary vascular tumour that was
rst identi ed and described by Zimmermann (1922) and accounts for
less than 1 per cent of vascular tumours. It stems from capillary pericytes (Kuhn and Rosai, 1969), which are contractile pericapillary cells.