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Background
This rare tumor must be distinguished from the histologically similar aggressive angiomyxoma. These tumors are well circumscribed tumors that characteristically occur in the pelviperineal region, commonly the vulva.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION AGE RANGE-MEDIAN Adult Majority females
PATHOGENESIS CHARACTERIZATION GENERAL Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases.
Granter SR, Nucci MR, Fletcher CD.
Department of Pathology, Brigham & Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Histopathology 1997 Jan;30(1):3-10 Abstract quote
Aggressive angiomyxoma is a distinctive soft tissue tumor associated with a high risk of local recurrence but lacks metastatic potential. This tumour occurs nearly exclusively in the soft tissues of the pelvis and perineum of adult women. The line of differentiation is not firmly established, but a fibroblastic/myofibroblastic origin has been proposed.
We report 16 new cases of aggressive angiomyxoma of the pelvic soft tissue in women. In all cases bundles of cells, most often adjacent to vessels, with histological features of smooth muscle cells were identified. In 11 of 14 cases the myoid bundles were immunoreactive for desmin; they were also positive for smooth muscle actin in 10 of 11 cases. In 13 of 14 cases lesional stromal cells showed immunoreactivity for desmin. Three cases showed areas with histological features similar to those of angiomyofibroblastoma of the vulva, thus representing previously undescribed morphological overlap between these two entities.
We conclude that aggressive angiomyxoma and angiomyofibroblastoma are related neoplasms in a spectrum of tumours showing myofibroblastic origin. Furthermore, the demonstration of immunoreactivity for desmin in aggressive angiomyxomas implies that this antibody is not helpful in discriminating between these two tumours, and the principal means of distinction remains histomorphological analysis.
LABORATORY/
RADIOLOGICCHARACTERIZATION RADIOLOGIC Perineal angiomyofibroblastoma: CT and MR findings with pathologic correlation.
Mortele KJ, Lauwers GJ, Mergo PJ, Ros PR.
Department of Radiology, University of Florida College of Medicine, Gainesville, USA.
J Comput Assist Tomogr 1999 Sep-Oct;23(5):687-9 Abstract quote
We present a case of a perineal angiomyofibroblastoma (AMFB) arising in the right perirectal fossa in a middle-aged woman, documented with CT and MRI.
Compounding the rarity of the entity, this case is unique as it is the first radiological report illustrating the CT and MR features of this recently clinicopathologically described neoplasm.
LABORATORY Angiomyofibroblastoma of the vulva: case report with immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature.
Sasano H, Date F, Yamamoto H, Nagura H.
Department of Pathology, Tohoku University School of Medicine, Sendai, Japan.
Pathol Int 1997 Sep;47(9):647-50 Abstract quote
A case of angiomyofibroblastoma of the vulva in a 49-year-old woman was examined.
The tumor measured 3 x 2.5 x 2 cm and appeared light gray to tan in color on the cut surface. Light microscopic examinations revealed that spindle or oval shaped tumor cells were arranged in loose edematous stroma with numerous thin-walled vessels.
Ultrastructurally, cell organellae were not well developed but intracytoplasmic filaments of intermediate size were abundant in the tumor cells. Desmin, CD34 and vimentin immunoreactivity were detected in almost all of the tumor cells. Both estrogen and progesterone receptors were diffusely expressed in the tumor, suggestive of the sex steroid-dependency of this tumor.
The KI-67 labeling index was less than 1% and the DNA content of the tumor cells, which was examined by image cytometry, demonstrated diploidy (DNA index = 0.97). These findings may reflect the quiescent or slow growing features of angiomyofibroblastoma.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION General VARIANTS FALLOPIAN TUBE Angiomyofibroblastoma arising from the fallopian tube.
Kobayashi T, Suzuki K, Arai T, Sugimura H.
Department of Obstetrics and Gynecology, Hamamatsu University School of Medicine, Hamamatsu-City, Shizuoka, Japan.
Obstet Gynecol 1999 Nov;94(5 Pt 2):833-4 Abstract quote
BACKGROUND: Angiomyofibroblastoma is a rare tumor that occurs most commonly in the vulva and vagina.
CASE: A 28-year-old woman, gravida 1, para 1, presented with secondary infertility. Pelvic ultrasound showed a multicystic mass that further imaging found to be septated and extended to the umbilicus. At laparotomy, a 25 x 14 x 4 cm mass originating from the left fallopian tube and the tube were excised. Pathologic examination confirmed an angiomyofibroblastoma of the fallopian tube.
CONCLUSION: Angiomyofibroblastomas can occur in the fallopian tube and should be differentiated from more aggressive angiomyxoma.
MALE Angiomyofibroblastoma-like tumor of the male genital tract:analysis of 11 cases with comparisons to female angiofibroblastoma and spindle cell lipoma
Laskin WB, etal.
Am J Surg Pathol 1998 Jan;22(1):6-16 Abstract quote
The clinicopathologic features and immunoprofile of 11 cases of an uncharacterized male genital tract tumor with features of vulvovaginal angiomyofibroblastoma (AMF) and spindle cell lipoma (male AMF-like tumor) are described.
The lesions presented as a mass involving the scrotum (six cases) or inguinal region (five cases) in males ranging in age from 39 to 88 years (median 57). The tumors were superficially located and well-marginated and ranged in size from 2.5 to 14 cm (approximate mean 7 cm). Microscopically, they were composed of tapered spindled cells proliferating between numerous small to medium-sized vessels. Epithelioid appearing stromal cells were a focal finding in four cases. Mitotic activity was minimal with no abnormal mitotic figures identified. Mild nuclear atypia was identified in two cases.
The tumors possessed an acid mucopolysaccharide-rich, finely collagenous stroma. A small quantity of intralesional fat was present in six cases. Tumor cells exhibited immunoreactivity for vimentin (seven of seven cases), progesterone receptor protein (five of seven cases), CD34 (four of eight cases), estrogen receptor protein (three of seven cases), desmin (three of eight cases), muscle-specific actin (three of eight cases), and smooth-muscle actin (two of eight cases) but not for S-100 protein.
One of seven patients with follow-up after simple excision had recurrent/persistent disease.
The male AMF-like tumor is a soft-tissue neoplasm of the male genital tract that shares clinicopathologic features and a proposed perivascular stem cell derivation with both the female angiomyofibroblastoma and spindle cell lipoma.
Angiomyofibroblastoma of the Male Perineum: An Unusual Location for a Rare Lesion.
Hlaing T, Tse G.
Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, Hong Kong.
Int J Surg Pathol 2000 Jan;8(1):79-82 Abstract quote
We report a case of angiomyofibroblastoma (AMF)-like tumor of the perineum in a 54-year-old man. The asymptomatic lesion measured 3 cm and appeared as a tan homogeneous mass.
Histologically, it appeared as a circumscribed nodular spindle cell proliferation with alternating cellular and hypocellular areas. The spindle cells exhibited minimal nuclear pleomorphism and scanty mitotic activity. Focally, some cells were epithelioid. Large blood vessels were present, with perivascular fibrosis. The spindle cells expressed vimentin, but not desmin, actin, S100, or CD34. These features were similar to those of AMF as initially reported in the vulva.
A perineal localion of this lesion in a male has not been reported in the literature, to the best of our knowledge.
Angiomyofibroblastoma of the male inguinal region.
Ito M, Yamaoka H, Sano K, Hotchi M.
Department of Pathology, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
Arch Pathol Lab Med 2000 Nov;124(11):1679-81 Abstract quote
We present a case of benign angiomyxoid tumor arising in the inguinal region of a 27-year-old man. The tumor was a gelatinous mass completely encapsulated by a thin fibrous capsule with no hemorrhage or necrosis.
Histologically, a proliferation of spindle cells as well as occasional pleomorphic cells was observed within the myxofibrous stroma, intermingled with abundant capillary-sized blood vessels. Immunohistochemical staining of the tumor demonstrated spindle, oval, and pleomorphic cells equally positive for vimentin, desmin, and CD34, but not for alpha-smooth muscle actin.
Based on these histologic and immunophenotypic features, we conclude that this angiomyxoid tumor of the male inguinal region is indistinguishable from the female angiomyofibroblastoma of the pelvic and perineal regions.
URETHRA Angiomyofibroblastoma of the female urethra.
Kitamura H, Miyao N, Sato Y, Matsukawa M, Tsukamoto T, Sato T.
Department of Urology, Sapporo Medical University School of Medicine, Japan.
Int J Urol 1999 May;6(5):268-70 Abstract quote
BACKGROUND: Angiomyofibroblastoma is a relatively recently described rare tumor of the superficial soft tissues. To date, 57 cases of angiomyofibroblastoma of the external genitalia in women have been reported.
METHODS/RESULTS: We describe a case of a 24-year-old woman who presented with the urinary stream flowing out in the posterior direction and whose diagnosis was a urethral tumor, angiomyofibroblastoma.
CONCLUSIONS: Angiomyofibroblastoma has a potential arising from the female urethra as well as other areas of the external genitalia.
VULVA Angiomyofibroblastoma of the vulva: case report.
Ustun C, Malazgirt Z, Kandemir B, Kocak I, Bolat I, Gumus S.
Department of Obstetrics and Gynaecology, Ondokuz Mays University, Samsun, Turkey.
Pathol Int 1998 Dec;48(12):964-6 Abstract quote
Angiomyxomas are a group of relatively rare mesenchymal myxoid tumors. Three types of angiomyxomas have been identified: superficial angiomyxoma, aggressive angiomyxoma and angiomyofibroblastoma. These tumors have a predilection for occurring in the perineum of middle-aged females and are often clinically diagnosed as Bartholin's cyst.
A case of a 42-year-old female with a perineal angiomyofibroblastoma mimicking a Bartholin's cyst is presented. The pathological and clinical characteristics of this tumor are discussed.
HISTOPATHOLOGY CHARACTERIZATION GENERAL Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma.
Fletcher CD, Tsang WY, Fisher C, Lee KC, Chan JK.
Department of Histopathology, St. Thomas's Hospital, Hong Kong.
Am J Surg Pathol 1992 Apr;16(4):373-82 Abstract quote
Aggressive angiomyxoma of pelvic soft parts is a rare lesion with a high risk of recurrence. We report 10 cases of angiomyofibroblastoma, a hitherto uncharacterized benign tumor of the vulva histologically mimicking aggressive angiomyxoma.
All patients had a vulval mass, often clinically diagnosed as a Bartholin's cyst. There was no recurrence after excision. The tumors were well circumscribed, measuring 0.5-12 cm in maximum dimension. They were characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels (predominantly of the capillary type) were irregularly distributed. Spindled, plump spindled, and oval stromal cells were aggregated around the blood vessels, sometimes forming solid compact foci, or were loosely dispersed in the hypocellular areas. Their nuclei were bland, but rare ones were enlarged and hyperchromatic in four cases. Some cells had abundant eosinophilic hyaline cytoplasm and eccentrically placed nuclei. Mitotic figures were absent or very sparse. Scattered throughout were thin, wavy strands or thick bundles of collagen. Mast cells were readily seen in eight cases. Immunohistochemically, the stromal cells were reactive for vimentin and desmin, but not cytokeratin, muscle-specific actin, alpha-smooth muscle actin, or S-100 protein.
Ultrastructural studies showed well-developed rough endoplasmic reticulum, Golgi apparatus, abundant intermediate filaments, and pinocytotic vesicles in the stromal cells.
Angiomyofibroblastoma can be distinguished from aggressive angiomyxoma by its circumscribed borders, much higher cellularity, more numerous blood vessels (which lack prominent hyalinization), frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation.
Angiomyofibroblastoma of the vulva: a clinicopathologic study of seven cases.
Hisaoka M, Kouho H, Aoki T, Daimaru Y, Hashimoto H.
Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Kitakyusyu, Japan.
Pathol Int 1995 Jul;45(7):487-92 Abstract quote
A clinicopathologic and immunohistochemical review was made of seven cases of angiomyofibroblastoma.
The patients were middle-aged women who had a slowly growing mass, measuring 1.5-6 cm in maximum dimension, located subcutaneously in the vulva. The tumors were well-demarcated and characterized by well-vascularized, alternating hypercellular and hypocellular edematous areas composed of bland, plump spindle- or oval-shaped stromal cells frequently aggregated around small blood vessels. An epithelioid appearance of the stromal cells was seen in two cases. Immunohistochemically, the stromal cells were consistently positive for vimentin and desmin, but negative for muscle specific actin, alpha-smooth muscle actin, myosin, cytokeratins, S-100 protein or von Willebrand factor. Ultrastructurally, the plump stromal cells had a small amount of peripherally located rough endoplasmic reticulum, numerous pinocytotic vesicles and abundant intermediate filaments, on which immunogold probes for desmin were localized, whereas fine filaments were few and there were no electron dense plaques.
Thus, while the proliferating stromal cells expressed an immunohistochemical profile of peculiar myoid differentiation, ultrastructural findings differed from those of smooth muscle cells or those seen in typical myofibroblasts. At 1-4 years after surgery, there was no evidence of recurrence.
Angiomyofibroblastoma of the vulva and vagina.
Nielsen GP, Rosenberg AE, Young RH, Dickersin GR, Clement PB, Scully RE.
James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, USA
Mod Pathol 1996 Mar;9(3):284-91 Abstract quote
Nine vulvar and three vaginal angiomyofibroblastomas from patients 23 to 71 years of age (mean, 46 yr) were analyzed. The tumors were well circumscribed and ranged from 0.9 to 11 cm (average, 4.7 cm) in maximal dimension.
On microscopic examination, they had hypercellular and hypocellular areas. The neoplastic cells were spindle-shaped, plasmacytoid, or epithelioid; a variable number were binucleated or multinucleated cells. A focal storiform pattern was present in one tumor, and, in one tumor, the neoplastic cells formed a collar around a central area of dense collagen. There was no significant nuclear atypia, and there was less than one mitotic figure per 10 high-power fields. The tumors contained small- to medium-sized blood vessels, which were characteristically thin walled and, occasionally, ectatic and branching. The stroma was edematous, separated collagen fibers and contained a variable number of inflammatory cells, especially lymphocytes and mast cells. Three vulvar tumors contained a variable amount of fat.
Ultrastructural study of three tumors showed intracytoplasmic, dilated, rough endoplasmic reticulum, moderate numbers of pinocytotic vesicles, and numerous filaments without dense bodies; rare intercellular rudimentary junctions were identified. Eleven of 11 tumors were immunoreactive for vimentin, 11 of 12 for desmin, three of 11 for muscle actin, one of 12 for smooth muscle actin, and four of 12 for CD34. There was no staining for factor XIIIa, keratin, S100 protein, Leu-7, glial fibrillary acidic protein, or CD68. Follow-up revealed no recurrences or metastases.
Angiomyofibroblastoma is a distinctive benign tumor that arises most commonly in the vulva and vagina and has a diverse histologic and immunohistochemical profile.
Vulval angiomyofibroblastoma. Clinicopathologic analysis of six cases.
Fukunaga M, Nomura K, Matsumoto K, Doi K, Endo Y, Ushigome S.
Department of Pathology, Jikei University School of Medicine, Tokyo, Japan.
Am J Clin Pathol 1997 Jan;107(1):45-51 Abstract quote
Clinicopathologic, immunohistochemical, ultrastructural, and flow cytometric analyses were performed on vulvar angiomyofibroblastomas in six women (ages 32 to 46 years; average age, 41.3 years). T
he tumors were all well circumscribed, and measured 2 to 9 cm in greatest dimension. Five tumors were clinically diagnosed as Bartholin's gland cysts. At histologic analysis, the tumors were characterized by alternating hypercellular and hypocellular edematous areas with abundant blood vessels. The hypercellular areas showed a perivascular proliferation of spindle-shaped and round cells, which often formed small nests or epithelioid arrangements, and a short fascicular pattern of spindle cells. The proliferating vessels were thin-walled and capillary-sized. Cellular atypia and mitotic figures were absent. Two tumors contained an adipose element, and one showed a prominent vascular proliferation reminiscent of a capillary hemangioma.
At immunohistochemical analysis, the tumor cells were strongly positive for vimentin and desmin. Some cells were weakly positive for HHF35 and alpha-smooth muscle actin. Ultrastructurally, one neoplasm showed fibroblastic differentiation. Flow cytometry revealed three tumors were DNA diploid and two were aneuploid. All tumors were excised, and none have recurred (average follow-up, 28.3 months).
These results indicate that angiomyofibroblastomas contain heterogeneous components.
Genital angiomyofibroblastoma. Comparison with aggressive angiomyxoma and other myxoid neoplasms of skin and soft tissue.
Ockner DM, Sayadi H, Swanson PE, Ritter JH, Wick MR.
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St Louis, Missouri 63110, USA.
Am J Clin Pathol 1997 Jan;107(1):36-44 Abstract quote
Angiomyofibroblastoma (AMFB) of the genital region is a relatively recently described tumor of the superficial soft tissues with a marked preference for female patients. Three cases of AMFB were reviewed, two of which involved adult men.
To further elucidate the pathologic features of AMFB, these three cases were compared with 10 cases of aggressive angiomyxoma (AA), a salient diagnostic alternative, and 28 cases of other myxoid tumors that may show morphologic similarities to these neoplasms. Conventional histologic and immunohistochemical features of AMFBs were compared with those of AA, myxoid leiomyoma, myxoid leiomyosarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, myxoid neurofibroma, and myxoid malignant peripheral nerve sheath tumor.
The ultrastructure of two of the three AMFBs also was analyzed. Genital AMFBs were circumscribed, partially myxoid proliferations that demonstrated considerable variation in cellular density. Neoplastic elements were bland cytologically and showed both fusiform and epithelioid profiles, with a tendency to concentrate around intralesional blood vessels. Mitotic activity and necrosis were absent, and the vessels assumed an arborizing configuration and were venule or capillary sized. In contrast, all other tumor types evaluated were infiltrative, cytologically atypical, or both. All AMFBs showed immunoreactivity for vimentin, desmin, actin, and estrogen receptor protein. These results were shared by most examples of AA and smooth muscle tumors as well, but were not seen in any other neoplasms in this study. Electron microscopic findings in cases of AMFB supported the presence of myofibroblastic differentiation in the tumor cells.
These results indicate that conventional morphologic analysis is paramount in the recognition of genital AMFB but that immunohistology may be helpful in a limited context in excluding other differential diagnoses. They also support the conclusion that AMFB, AA, and superficial smooth muscle tumors have similar morphotypes and immunohistologic attributes regardless of their origin in men or women.
Clinicopathologic analysis of angiomyofibroblastoma of the female genital tract.
Wang J, Sheng W, Tu X, Shi D, Zhu X, Zhang R.
Department of Pathology, Cancer Hospital, Shanghai Medical University, 399 Lingling Road, Shanghai 200032, China.
Chin Med J (Engl) 2000 Nov;113(11):1036-9 Abstract quote
OBJECTIVE: To study the clinicopathologic features and immunoprofile of angiomyofibroblastoma (AMF) with emphasis on differential diagnosis.
METHODS: Seven vulvar, two vaginal and one perineal angiomyofibroblastomas were evaluated by light microscopy and immunohistochemistry. The immunohistochemical studies were performed on paraffin-embedded sections using the standard ABC method with a panel of antibodies.
RESULTS: Angiomyofibroblastoma predominantly occurred in middle-aged women in the genital region, especially in the superficial area of the vulva. Clinically, most of the tumors presented as slowly growing painless masses and were often diagnosed as Bartholin's gland cysts. Histologically, the tumors were all well-circumscribed and characterized by alternating hypocellular and hypercellular areas with abundant thin-walled blood vessels. The tumor cells were bland and spindle-shaped or epithelioid and tended to concentrate around the vessels or cluster in small nests. Immunohistochemically, the tumor cells expressed vimentin and estrogen receptor protein in all 10 cases and desmin in 9 cases. Three cases showed weak or focal immunoreactivity to alpha-smooth muscle actin and muscle specific actin. All ten patients were treated by local excision. Follow-up showed a benign course with no signs of recurrence.
CONCLUSIONS: Angiomyofibroblastoma is a distinctive neoplasm that has a propensity to occur in the female genital tract. Recognition of this entity is important to avoid misdiagnosis with other vulvar angiomyxoid neoplasms.
Angiomyofibroblastoma of the vulva: report of a case.
Tochika N, Takeshita A, Sonobe H, Matsumoto M, Kobayashi M, Araki K.
Department of Surgery, Kochi Medical School, Okocho, Japan.
Surg Today 2001;31(6):557-9 Abstract quote
A 45-year-old woman was first seen by us 2 years after becoming aware of a slightly painful subcutaneous mass in her left vulva. The mass was 7.5 x 3.0 cm in size, well circumscribed, mobile, and rubbery. It was at first clinically considered to be a benign tumor.
Microscopically, the resected mass was composed of spindle or polygonal tumor cells which were cellularly or hypocellularly arranged with perivascular accentuation in a mucoid or fibrocollagenous background.
Immunohistochemically, myxoid tumor cells were positive for vimentin but not for alpha-smooth muscle actin, CD34, CD31, desmin, or S-100 protein. The tumor was diagnosed as an angiomyofibroblastoma (AMBF), based on the typical findings of histology and immunohistochemistry. There are many histological types of vulvar tumors, and establishing a preoperative diagnosis is difficult in many patients.
Rapid intraoperative pathological diagnosis should be performed if possible, considering the possibility of diseases such as AMFB and aggressive angiomyxoma (AAM). When AAM is suspected, the peripheral tissues should also be resected to prevent recurrence.
VARIANTS LIPOMATOUS Angiomyofibroblastoma of the female genital tract: analysis of 17 cases including a lipomatous variant.
Laskin WB, Fetsch JF, Tavassoli FA.
Department of Pathology, Northwestern University Medical School, Chicago, IL 60611-3053, USA.
Hum Pathol 1997 Sep;28(9):1046-55 Abstract quote
The clinicopathological and immunohistochemical profile of 17 cases of angiomyofibroblastoma (AMF) arising in the genital tract of females is reported.
The lesions usually presented as painless masses and were located in the superficial vulvar region (15 cases), canal of Nuck (one case), and perineum (one case) in women ranging in age from 38 to 60 years (median, 46 years). The tumors were well delineated and ranged in size from 2 to 8 cm in greatest dimension.
Microscopically, they were composed of spindled and epithelioid mesenchymal cells arranged in cords and nests preferentially arrayed around numerous small to medium-sized vessels. Mitotic activity ranged from 0 to 7 mitoses per 50 high-power fields (HPF) with no abnormal mitotic figures. Minimal nuclear atypia was appreciated.
Intralesional fat was present in 12 cases and in two of these cases constituted most of the tumor (lipomatous variant of AMF). Tumor cells expressed vimentin (five of five cases), estrogen receptor protein (six of six cases), progesterone receptor protein (five of six cases), desmin (six of eight cases), CD34 (one of six cases), and smooth muscle actin (one of seven cases). None of the eight women with follow-up of up to 25 years (mean, 7.8 years) after simple excision developed a recurrence.
This study confirms the benign nature of AMF, broadens its morphological spectrum to include a lipomatous variant, and proposes an origin from a perivascular stem cell that is capable of myofibroblastic and fatty differentiation.
MITOTICALLY ACTIVE Angiomyofibroblastoma of the vulva: a mitotically active variant?
Takeshima Y, Shinkoh Y, Inai K.
Second Department of Pathology, Hiroshima University School of Medicine, Japan.
Pathol Int 1998 Apr;48(4):292-6 Abstract quote
A case of angiomyofibroblastoma in a 48-year-old woman is reported. The tumor occurred as a left vulval mass and was treated by simple excision. It was located in the subcutaneous tissue of the left vulva and was well circumscribed, measuring 2.8 x 2.7 x 2.5 cm.
Microscopically, the tumor was composed of hypocellular and cellular areas with well-developed small vessels. Spindle or polygonal cells were arranged with perivascular accentuation in an edematous or fibrocollagenous background. Some spindle-shaped or polygonal stromal cells were also arranged in epithelioid nests.
In some areas, mitoses were frequent (maximum 3/10 high-power field). Immunohistochemically, the stromal cells were positive for vimentin and desmin, but negative for alpha-smooth muscle actin, S-100, neurofilament, estrogen receptor, progesterone receptor, CD31 and CD34. The average labeling index of Ki-67 in stromal cells was 3.1%. Ultrastructural analysis demonstrated that the stromal cells adhered with primitive junctions and contained intermediate filaments with no focal density in the cytoplasm.
These findings were consistent with angiomyofibroblastoma, although previously reported cases did not show so many mitoses. Therefore, this case was suggested to be a mitotically active variant.
SPECIAL STAINS/
IMMUNO-HISTOCHEMISTRYCHARACTERIZATION Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study.
Bigotti G, Coli A, Gasbarri A, Castagnola D, Madonna V, Bartolazzi A.
Department of Pathology, Catholic University of Sacred Heart, Rome, Italy.
Pathol Res Pract 1999;195(1):39-44 Abstract quote
We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation.
Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type IV, CD68 and S-100. The stromal cells of AAM were negative for laminin.
These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix.
It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract.
ELECTRON MICROSCOPY Angiomyofibroblastoma of the vulva: case report with immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature.
Sasano H, Date F, Yamamoto H, Nagura H.
Department of Pathology, Tohoku University School of Medicine, Sendai, Japan.
Pathol Int 1997 Sep;47(9):647-50 Abstract quote
A case of angiomyofibroblastoma of the vulva in a 49-year-old woman was examined. The tumor measured 3 x 2.5 x 2 cm and appeared light gray to tan in color on the cut surface. Light microscopic examinations revealed that spindle or oval shaped tumor cells were arranged in loose edematous stroma with numerous thin-walled vessels.
Ultrastructurally, cell organellae were not well developed but intracytoplasmic filaments of intermediate size were abundant in the tumor cells. Desmin, CD34 and vimentin immunoreactivity were detected in almost all of the tumor cells. Both estrogen and progesterone receptors were diffusely expressed in the tumor, suggestive of the sex steroid-dependency of this tumor. The KI-67 labeling index was less than 1% and the DNA content of the tumor cells, which was examined by image cytometry, demonstrated diploidy (DNA index = 0.97).
These findings may reflect the quiescent or slow growing features of angiomyofibroblastoma.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES AGGRESSIVE ANGIOMYXOMA CELLULAR ANGIOFIBROMA Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma.
Nucci MR, Granter SR, Fletcher CD.
Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts 02115, USA.
Am J Surg Pathol 1997 Jun;21(6):636-44 Abstract quote
Four cases of a distinctive soft-tissue tumor of the vulva are described. They were characterized by occurrence in middle-aged women (39-50 years), small size (< 3 cm), and a usually well-circumscribed margin.
The preoperative clinical diagnosis was that of a labial or Bartholin gland cyst in three of the four cases. The microscopic appearance was remarkably consistent and was characterized by a cellular neoplasm composed of uniform, bland, spindled stromal cells, numerous thick-walled and often hyalinized vessels, and a scarce component of mature adipocytes. Mitotic activity was brisk in three cases (up to 11 mitoses per 10 high power fields). The stromal cells were positive for vimentin and negative for CD34, S-100 protein, actin, desmin, and epithelial membrane antigen, suggesting fibroblastic differentiation. Two patients with follow-up showed no evidence of recurrence.
The differential diagnosis of this distinctive tumor includes aggressive angiomyxoma, angiomyofibroblastoma, spindle cell lipoma, solitary fibrous tumor, perineurioma, and leiomyoma.
The designation of "cellular angiofibroma" is chosen to emphasize the two principal components of this tumor: the cellular spindle cell component and the prominent blood vessels.
Extravulvar subcutaneous cellular angiofibroma.
Garijo MF, Val-Bernal JF.
Department of Anatomical Pathology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain.
J Cutan Pathol 1998 Jul;25(6):327-32 Abstract quote
Cellular angiofibroma is a rare distinctive mesenchymal neoplasm of the vulva or perineal region.
We report here one unique extravulvar case. A 43-year-old woman presented with an asymptomatic tumor, 7 cm in diameter, located in the subcutaneous tissue of the chest below the left submammary sulcus.
Histologically, the lesion was composed of uniform spindled stroma cells, numerous thick-walled vessels, and scarce mature adipocytes. An additional feature was the presence of prominent perivascular lymphoid aggregates. The stromal cells were positive for vimentin and negative for CD34 and muscle, epithelial, myoepithelial, or neural markers. Although nasopharyngeal angiofibroma or the group of acral angiofibromas have a concurrent heading, cellular angiofibroma should not be mistaken with them.
Differential diagnosis of this distinctive tumor especially includes aggressive angiomyxoma, angiomyofibroblastoma, superficial angiomyxoma, vascular myxolipoma, and other tumors with spindle cells reminiscent of those in angiofibroma.
SUPERFICIAL CERVICOVAGINAL MYOFIBROBLASTOMA Superficial cervicovaginal myofibroblastoma: fourteen cases of a distinctive mesenchymal tumor arising from the specialized subepithelial stroma of the lower female genital tract.
Laskin WB, Fetsch JF, Tavassoli FA.
Department of Pathology, Northwestern University Medical School, Chicago, IL, USA.
Hum Pathol 2001 Jul;32(7):715-25 Abstract quote
The clinicopathologic features and immunohistochemical profiles of 14 cases of a distinctive mesenchymal tumor that arises in the superficial lamina propria of the cervix and vagina and is histologically distinguishable from mesodermal (fibroepithelial) stromal polyp, including the cellular (pseudosarcomatous) variant, angiomyofibroblastoma, aggressive angiomyxoma, and other well-recognized lesions that occur in this location, are described.
The lesions presented as a polypoid (n = 10) or nodular (n = 4) mass in the vagina (n = 12) or cervix (n = 2) of women ranging in age from 40 to 74 years (median, 58 years). The tumors were subepithelial in location, were well circumscribed, and ranged in size from 1 to 6.5 cm. (mean, 2.7 cm).
Microscopically, the process was moderately to highly cellular and composed of relatively bland spindled and stellate-shaped mesenchymal cells embedded in a finely collagenous stroma that was punctuated by myxoid and edematous foci in 9 cases. The lesions characteristically had a multipatterned architecture with tumor cells focally assuming a lacelike/sievelike growth pattern in the more stroma-rich areas of the tumor and a vague fascicular growth pattern in the more cellular foci. Mitotic activity was minimal, and no atypical mitotic figures were identified.
The tumors were immunoreactive (in decreasing order of relative strength) for vimentin (5 of 5 cases), estrogen (10 of 10 cases), and progesterone (10 of 10 cases) receptors, desmin (13 of 13 cases), CD34 (11 of 13 cases), alpha-smooth muscle actin (5 of 11 cases), and muscle-specific actin (2 of 8 cases). The desmin and CD34 antibodies highlighted the interconnecting, dendritic processes associated with many of the tumor cells. No immunoreactivity was detected for S100 protein, epithelial membrane antigen, or keratins. Follow-up data for 11 patients (range, 1 to 20 years; median, 4 years) showed no recurrence or metastasis after local excision.
The term "superficial cervicovaginal myofibroblastoma" is proposed because it reflects the distinguishing features of this benign, relatively site-specific mesenchymal tumor. The process probably arises as a neoplastic proliferation of hormonally responsive mesenchymal cells native to the unique subepithelial stromal layer normally found through the endocervix and vulva of adult women.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS Most cases are benign and slow growing MALIGNANT TRANSFORMATION Angiomyofibroblastoma of the vulva with sarcomatous transformation ("angiomyofibrosarcoma").
Nielsen GP, Young RH, Dickersin GR, Rosenberg AE.
Massachusetts General Hospital, and the Department of Pathology, Harvard Medical School, Boston 02114, USA.
Am J Surg Pathol 1997 Sep;21(9):1104-8 Abstract quote
We report on a locally recurrent vulvar tumor in an 80-year-old woman that we believe represents the first example of malignant transformation of an angiomyofibroblastoma.
The tumor was predominantly a typical angiomyofibroblastoma, composed of epithelioid or oval cells with eosinophilic cytoplasm that tended to cluster in small groups and around blood vessels. These areas merged imperceptibly with a high-grade sarcoma that resembled a myxoid malignant fibrous histiocytoma. The tumor cells in the benign areas were diffusely immunoreactive for vimentin; many cells were positive for smooth muscle actin, and focal positivity for muscle actin and desmin was observed.
The tumor cells in the sarcomatous areas were diffusely positive for vimentin, but negative for smooth muscle actin, muscle actin, and desmin. No staining for keratin, S-100 protein, or CD34 was noted. Ultrastructural examination of the sarcomatous area showed that the cells had the features of fibroblasts. All previously reported cases of angiomyofibroblastoma have exhibited banal histologic features and have behaved in a benign fashion.
This case shows that these tumors may rarely be associated with a malignant component, and the designation "angiomyofibrosarcoma" may be appropriate in such cases.
TREATMENT Simple excision Am J Surg Pathol 1992;16:373-382
Am J Clin Pathol 1997;107:36-44
Am J Clin Pathol 1997;107:45-51
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