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360

A.C. Tsili

 

 

a

b

Fig. 17  FIGO stage IVA cervical carcinoma in 50-year- old woman. Sagittal (a) T2WI and (b) dynamic contrastenhanced subtracted MR image (early phase) show a bulky heterogeneous mass that arises from the cervix and extends to the lower third of the vagina. The tumor appears with T2 signal mainly similar to that of normal myome-

trium, enhancing inhomogeneously, less than myometrium after gadolinium administration, with areas of necrosis and a small amount of air. The mass invades the posterior wall of the urinary bladder (long arrow). Obstruction of the endocervical canal by the neoplasm causes distention of the endometrial cavity (arrow)

with vaginal metastases and therefore the prognosis is extremely poor.

Seventy-five percent of squamous vaginal metastases arise from the cervix and 14% from the vulva. Of the vaginal metastases that are adenocarcinomas, 92.5% of lesions in the upper third and anterior wall arise from the upper genital tract, while 90% of lesions in the lower third and posterior wall arise from the gastrointestinal tract. The overall accuracy of MRI in assessing vaginal metastases has been reported as 92%. The MRI features of vaginal metastases mimic the MRI features of the primary tumor, usually detected with low to intermediate T1 signal and intermediate to high T2 signal (Walker et al. 2011; Griffin et al. 2010; Parikh et al. 2008).

6.2\ Vulvar Malignancies

6.2.1\ Vulvar Carcinoma

Vulval carcinoma is rare, accounting for 4% of all gynecologic malignancies. The disease has a bimodal distribution, with approximately 66% of cases seen over the age of 70 years and fewer

than 20% occurring in women younger than 50 years (Griffin et al. 2010; Hosseinzadeh et al. 2012; Lee et al. 2011; Ssi-Yan-Kai et al. 2015; Sohaib et al. 2002). Human papilloma virus-pos- itive tumors occur in younger age, may be multifocal, and show an association with vulvar intraepithelial neoplasia. Patients may present with a palpable mass, bleeding due to ulceration, pruritus, pain, and discharge.

Vulval cancer involves the labia in two-thirds of cases. The clitoris and Bartholin glands are less commonly involved. It is locally infiltrative and may extend to the urethra, anorectum, and vagina, and rarely to the bladder. It typically spreads to the ipsilateral superficial inguinofemoral lymph nodes, followed by the deep inguinofemoral lymph nodes, before pelvic lymph nodes. Rarely, it extends beyond the pelvis (Griffin et al. 2010; Hosseinzadeh et al. 2012; Lee et al. 2011; Ssi-Yan-Kai et al. 2015; Sohaib et al. 2002).

More than 85% are squamous cell carcinomas; other primary histologic types include adenocarcinoma, sarcoma, Bartholin gland cancer, basal cell cancer, and extramammary Paget disease. The most important prognostic

Vagina and Vulva

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c

Fig. 18  Vaginal metastases from endometrial carcinoma (skip lesions) in an 80-year-old woman. Sagittal (a) T2WI and (b) dynamic contrast-enhanced subtracted MR image (early phase) demonstrate large heterogeneous masses replacing the uterus (arrowhead) and the vagina (arrow).

The neoplasms have similar imaging findings, detected mainly with intermediate T2 signal and heterogeneous contrast enhancement. (c) Transverse ADC map at the level of vaginal mass shows tumor with low signal intensity (arrow), due to restricted diffusion

a

b

Fig. 19  Vaginal metastasis from colon adenocarcinoma in 82-year-old woman. Sagittal T2WI (a) and (b) fat-sup- pressed TIW1 after gadolinium administration depict heterogeneous vaginal mass (arrow), inhomogeneously

enhancing. (c) Axial fat-saturated TIW1 after gadolinium administration shows left inguinal metastatic lymphadenopathy (arrowhead)

362

A.C. Tsili

 

 

c

Fig. 19  (continued)

Table 3  FIGO staging for vulvar cancer

FIGO

Definition

IA

Tumor confined to vulva or perineum, ≤2 cm

 

in size with stromal invasion ≤1 mm, negative

 

nodes

IB

Tumor confined to vulva or perineum, >2 cm

 

in size or with stromal invasion >1 mm,

 

negative nodes

IITumor of any size with adjacent spread (1/3 lower urethra, 1/3 lower vagina, anus), negative nodes

IIIA

Tumor of any size with positive

 

inguinofemoral lymph nodes

 

(1)

1 lymph node metastasis greater than or

 

equal to 5 mm

 

(2)

1–2 lymph node metastasis(es) of less than

 

5 mm

IIIB

(1)

2 or more lymph node metastases greater

 

than or equal to 5 mm

 

(2)

3 or more lymph node metastases less than

 

5 mm

IIIC

Positive node(s) with extracapsular spread

 

 

 

IVA

(1)

Tumor invades other regional structures

 

(2/3 upper urethra, 2/3 upper vagina), bladder

 

mucosa, rectal mucosa, or fixed to pelvic bone

 

(2)

Fixed or ulcerated inguinofemoral lymph

 

nodes

IVB

Any distant metastasis including pelvic lymph

 

nodes

factors­ determining­ survival include tumoral size, depth of invasion, and presence of lymph node metastases. The FIGO staging classification (Tan et al. 2012) is presented in Table 3. Patients with negative­ groin nodes have a 90% survival, compared to 50% survival with positive nodes.

Recurrences are usually seen within 2 years after initial treatment, mostly in the vulva (57%), groin (22%), pelvis (4%), or distant sites (23%) (Griffin et al. 2010; Hosseinzadeh et al. 2012; Lee et al. 2011; Ssi-Yan-Kai et al. 2015; Sohaib et al. 2002).

MRI is the modality of choice to allow proper tumoral delineation, evaluation of the local extent of vulvar cancer, and its relationship to adjacent structures, to aid in surgical planning and to reduce surgical morbidity (Griffin et al. 2010; Hosseinzadeh et al. 2012; Lee et al. 2011; Ssi- Yan-Kai et al. 2015; Sohaib et al. 2002). The tumor appears as a solid mass with nonspecific low T1 signal, intermediate-to-high T2 signal, and variable contrast enhancement (Figs. 20 and 21). The reported sensitivity and specificity of MRI in detecting metastatic lymphadenopathy in patients with vulvar carcinoma is reported 52–86% and 82–85%, respectively (Lee et al. 2011). Location, size, shape, and internal architecture on CT or MRI should be assessed to diagnose lymph node involvement. Size criteria show suboptimal accuracy; almost 60% of metastatic lymph nodes are smaller than 5 mm in diameter. Loss of the fatty hilum and a more round rather than elongated shape are features suggestive of tumor involvement. A decrease in central enhancement, indicating necrosis, and T2 signal heterogeneity on MRI are features suspicious for metastatic infiltration (Lee et al. 2011).

6.2.2\ Melanoma

Melanoma is the second most common vulval malignancy, accounting for 5% of malignant vulval neoplasms. MRI features are similar to those described for vaginal melanoma (Griffin et al. 2010; Hosseinzadeh et al. 2012; Ssi-Yan-Kai et al. 2015).

6.2.3\ Lymphoma

Primary or secondary non-Hodgkin lymphoma (NHL) of the female genital tract is rare, more often seen as a manifestation of systemic disease. Rarely, NHL may arise primarily in gynecologic organs, with the vulva being the least commonly affected organ. Primary NHL of the vulva is aggressive, mostly occurring in the elderly. The final diagnosis is made through percutaneous core or