Soft Tissue Neoplasms of the Lower Female Genital Tract

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Soft Tissue Neoplasms of the Lower Female Genital
Tract

• Terence Rollason
• Birmingham Womens Hospital

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Tumour Types

• Relatively site specific e.g.
• Fibroepithelial stromal polyps
• Angiomyofibroblastoma
• Aggressive angiomyxoma
• Cellular angiofibroma
• Widespread distribution e.g.
• Leiomyoma
• Lipoma
• Dermatofibroma etc.

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Myxoid Vulval Tumours

• Aggressive angiomyxoma
• Angiomyofibroblastoma
• Fibroepithelial polyps
• Myxoid and myxohyaline SMTs
• Superficial angiomyxoma
• Myxoid neurofibroma

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Aggressive Angiomyxoma

• Vulval or paravaginal. Mean age 30s. Extend
  into pelvis, particularly on recurrence
• Slow growth with multiple recurrences. No
  metastases ( a single case of apparent pulmonary
  metastasis described)
• Large, gelatinous mass with poorly defined margin

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Aggressive Angiomyxoma

• Bland, spindle and stellate cells. Vascular with
  medial hypertrophy in arteries
• No mitoses
• Low cellularity and fibrillary background
• Myoid bundles around vessels
• Mast cells may be numerous
• Vimentin, actin and sometimes desmin positive. ER
  PR ve (?useful in Rx).

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Aggressive Angiomyxoma

• Therapy in the past has been surgical but there
  may be a role for GnRH analogues
• There appears to be a chromosome 12
  re-arrangement in AA and this may allow diagnosis
  via a specific marker - HMGA2

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Non-Neoplastic Mimics of Aggressive Angiomyxoma

• Prolapsed fallopian tube with angiomyofibroblastic
  response (Michal et al, 2000). Suggested as a
  mimic also for angiomyofibroblastoma and
  superficial myofibroblastoma
• Vulval hypertrophy with oedema in quadriplegics
  etc. (Vang et al, 2001)

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Angiomyofibroblastoma

• Circumscribed and lt5cm diameter usually
• Relatively superficial compared to aggressive
  angiomyxoma
• Often thought to be Bartholins cysts

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Angiomyofibroblastoma

• Ovoid, round or spindled cells in loose stroma
  with numerous capillaries and whispy collagen.
  Adipose tissue may be seen, particularly near
  margins.
• Cellular aggregates and solid areas may give
  zonal pattern and epithelioid differentiation may
  be present.
• Plasmacytoid cells typical near vessels. Mast
  cells may be abundant and giant cells may be
  seen.
• Desmin positive, usually actin negative. ER PR
  positive

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Angiomyofibroblastoma

• Perivascular cell aggregates and desmin
  positivity may also be seen in aggressive
  angiomyxoma - disease continuum exists but those
  in middle may behave more like AA
• Mitotically active and lipomatous variants
  described
• One report of malignant transformation to sarcoma
  and a small series of cases of co-existence with
  sarcomas described

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Fibroepithelial Stromal Polyps

• Any age, commoner in vagina, often found in
  pregnancy when vaginal may be multiple
• Usually solitary on vulva and may be large (up to
  12 cm)
• Thinned or thickened epithelium, may be
  frond-like
• Spindled or stellate stroma often with
  pleomorphic stromal cells and wreath-like
  multinucleate cells. Fibrovascular core. Desmin,
  ER PR ve

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Fibroepithelial Stromal Polyps

• May be very pleomorphic (particularly in
  pregnancy) or cellular stroma and mitotic rate
  may be high. Abnormal mitoses may be seen.
• Helpful features in diff. diagnosis with sarcoma
  are central position of most bizarre or cellular
  areas, fibrovascular core.
• Lack of clear demarcating zone between epithelium
  and stroma helps in differential diagnosis with
  AMF and SMF
• Vaginal rhabdomyoma similar but with striated
  muscle cells. Mixed tumours also occur in
  vagina

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Superficial Angiomyxoma (Cutaneous Myxoma)

• Usually head, neck and trunk
• 2-4th decades
• Slow growing, margins may be well or poorly
  defined
• Involves dermis and subcutis
• Multilobulate pattern with myxoid nodules

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Superficial Angiomyxoma (Cutaneous Myxoma)

• Stellate fibroblasts
• Numerous thin walled vessels
• Scattered inflammatory cells including
  neutrophils
• One third show epithelial element - squamous or
  basaloid
• Vimentin, CD34, SMA ve. May be S100 ve. Desmin
  -ve
• Perhaps one third recur

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Myxoid Neurofibroma

• Buckled / wavy nuclei
• S100 positivity and desmin negativity
• Lack of large and thick walled vessels
• May be well demarcated or irregularly
  interdigitating
• Even sarcomas may be bland - careful search for
  mitoses

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Vulvar Smooth Muscle Tumours

• Leiomyomas here are dissimilar to pilar
  (cutaneous) sites
• Larger
• More frequently myxoid, hyaline and epithelioid
• Leiomyosarcomas are the most common sarcomas of
  the vulva but make up only 1-2 of vulval
  malignancies

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Vulvar Smooth Muscle Tumours

• What features indicate malignancy? Criteria for
  uterine SMTs cannot be used at this site.
• Nielsen et al suggest any three from
• 5 or more mitoses per 10HPF
• Infiltrative margins
• 5cm or more diameter
• moderate to severe atypia
• Any 2 atypical leiomyoma
• Based on only 19 cases with 4 recurrences and 1
  death

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Vulvar Smooth Muscle Tumours

• Tavassoli - gt5cm diameter and gt5mitoses per 10
  HPF predicts recurrence
• Nucci Fletcher
• any mitotic activity, pleomorphism or
  infiltration risk of recurrence, often after
  several years.
• Recurrences tend to be more pleomorphic. They
  termed tumours with any of these features
  atypical SMT.

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Myxoid and Myxohyaline SMT

• May be diffusely myxoid or show skein-like
  arrays. Look for foci of classical fascicular
  SMT
• Myxohyaline show packets of spindled cells
  separated by myxoid or hyaline zones
• Mitotic activity low and little or no
  pleomorphism
• None in literature described as malignant at this
  site

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Superficial Cervical / Vaginal Myofibroblastoma

• 14 cases originally described (Laskin et al,
  2001) in cervix and vagina. Further 12 cases with
  a few in vulva
• Arise in superficial subepithelial tissues
• Polypoid or nodular mass
• Wide age range

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Superficial Cervical / Vaginal Myofibroblastoma

• Moderately to highly cellular with Grentz zone
• Bland spindled and stellate cells
• Fine collagenous stroma with myxoid and
  oedematous zones
• Multipatterned - lacelike, sievelike and
  fascicular
• Vimentin, ER/PR, desmin and CD34 ve, SMA occ.
  ve
• No recurrences or metastases

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Vulvar Cellular Angiofibroma

• Middle aged women, almost always vulval but
  described in inguinal and scrotal area
• Benign, well circumscribed, superficial, usually
  lt5cm diameter. Only one local recurrence
  described.
• Uniform, bland, spindled stromal cells with
  numerous thick-walled, often hyalinized vessels.
  Moderately cellular.

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Vulvar Cellular Angiofibroma

• Mitoses may be frequent
• Cells arranged in short, intersecting fascicles
  with fine collagen bundles throughout
• Scant, marginal adipose tissue
• Desmin -ve. Occasionally CD34 ve. Actin -ve.
  ER/PR ve.

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Vulval Liposarcoma / Atypical Lipoma

• Rare tumours, usually seen in middle aged
• Usually well differentiated liposarcoma /
  atypical lipoma
• Variation in adipocyte size, atypia, occasional
  lipoblasts

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Vulval Liposarcoma / Atypical Lipoma

• Vulval tumours may be more varied in appearance
  than those at other sites with spindle cells,
  bivacuolate lipoblasts and adipocytes of varied
  size
• May be confused with spindle cell lipoma
• Only one of six in recent series recurred (Nucci
  Fletcher)

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Lipoblastoma-like Tumour

• 13-38 years of age
• 35-100mm
• Well circumscribed and clinically appear benign
  to date
• Lobulated with myxoid appearance and striking
  chicken-wire vascular pattern
• Spindle cell tumours but with signet-ring
  lipoblasts
• IHC showed only vimentin positivity

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Pre-pubertal Vulval Fibroma

• Mainly labia majora of 4-12 year old girls
• Ill defined, submucosal or subcutaneous
  hypocellular tumours composed of spindle shaped,
  bland cells
• Collagenous, oedematous or myxoid stroma
• Diffusely infiltrate other normal structures
• CD34 ve, desmin and SMA negative
• May recur locally and early but no metastases

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Vulvar SCC with Fibromyxoid Stromal Reaction

• Relatively recently described and prognostic
  importance not clear
• Suggestion of poorer prognosis than SCC NOS

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Other Reports

• Nodular fasciitis
• Solitary fibrous tumour
• Fibromatosis
• Post-operative spindle cell nodule
• Spindle cell lipoma
• Mammary-type myofibroblastoma