Aggressive Angiomyxoma of the Lower Female Genital Tract: A Review of the MITO Rare Tumors Group.

Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30-40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases.

Aggressive Angiomyxoma of the Lower Female Genital Tract in Pregnancy: A Review of the MITO Rare Tumors Group.

Deep (aggressive) angiomyxoma of the lower genital tract is a rare malignancy affecting women of reproductive age. Being a hormone-sensitive tumor, its growth is particularly benefitted during pregnancy. Surgical excision with complete resection is indicated, even if a wait-and-see approach can be considered until delivery, to avoid destructive surgeries. The mode of delivery is to be evaluated based on the location and size of the neoplasm; vaginal delivery is not contraindicated, as long as the tumor does not obstruct the birth canal. Positive surgical margins are the most important prognostic factor for recurrence. Adjuvant therapy with gonadotropin-releasing hormone analogues may be proposed after pregnancy, in the case of non-radical surgery. Despite the high local relapse rate, the outcomes for mother and child are favorable. Since recurrences can occur after many years, the patient should be included in long-term follow-up.

Preoperative Tumor Texture Analysis on MRI for High-Risk Disease Prediction in Endometrial Cancer: A Hypothesis-Generating Study.

OBJECTIVE: To develop and validate magnetic resonance (MR) imaging-based radiomics models for high-risk endometrial cancer (EC) prediction preoperatively, to be able to estimate deep myometrial invasion (DMI) and lymphovascular space invasion (LVSI), and to discriminate between low-risk and other categories of risk as proposed by ESGO/ESTRO/ESP (European Society of Gynaecological Oncology-European Society for Radiotherapy & Oncology and European Society of Pathology) guidelines. METHODS: This retrospective study included 96 women with EC who underwent 1.5-T MR imaging before surgical staging between April 2009 and May 2019 in two referral centers divided into training (T = 73) and validation cohorts (V = 23). Radiomics features were extracted using the MODDICOM library with manual delineation of whole-tumor volume on MR images (axial T2-weighted). Diagnostic performances of radiomic models were evaluated by area under the receiver operating characteristic (ROC) curve in training (AUCT) and validation (AUCV) cohorts by using a subset of the most relevant texture features tested individually in univariate analysis using Wilcoxon-Mann-Whitney. RESULTS: A total of 228 radiomics features were extracted and ultimately limited to 38 for DMI, 29 for LVSI, and 15 for risk-classes prediction for logistic radiomic modeling. Whole-tumor radiomic models yielded an AUCT/AUCV of 0.85/0.68 in DMI estimation, 0.92/0.81 in LVSI prediction, and 0.84/0.76 for differentiating low-risk vs other risk classes (intermediate/high-intermediate/high). CONCLUSION: MRI-based radiomics has great potential in developing advanced prognostication in EC.

"Intestinal-Type" Vulvar Adenocarcinoma: A Review of the MITO Rare Tumors Group.

Intestinal-type adenocarcinoma (VAIt) represents a sporadic variant of vulvar carcinoma. It appears frequently localized to epithelial glands in the vulvar region, and it probably derives from cloacal remnants persisting in the adult. We performed a systematic review of the limited cases reported in the literature, with the intent to assess the specific peculiarities of this rare neoplasia and to state consistent management recommendations. The principal histological VAIt characteristic is that it resembles mucinous colonic carcinomas. Therefore, immunohistochemical workup, with different tumor markers including CK20, CDX2, and CK7 staining, is needed. To confirm vulvar origin, a thorough diagnostic, and radiological examination is required to rule out other primary malignancies. The gold standard of treatment for VAIt is surgery, with local excision with tumor-free margins. Lymph node staging is an option advised if the tumor size is >2 cm or if lymph node metastases are suspected on imaging. On the other hand, the role of neoadjuvant therapy is still in doubt, but a good response to adjuvant chemotherapy treatments has been described in both advanced and recurrent diseases. Sometimes, VAIt behavior can be unpredictable, with relapses even after many years, so more experiences and longer follow-up periods are needed to elucidate the best therapeutic management and its long-term prognosis.

Preoperative Serum Human Epididymis Protein 4 Levels in Early Stage Endometrial Cancer: A Prospective Study.

OBJECTIVE: The aim of the study was to evaluate the prognostic value of human epididymis protein 4 (HE4) and cancer antigen 125 markers with pathological prognostic factor to complete the preoperative clinical panel and help the treatment planning. METHODS: This prospective multicenter study was conducted in 2 gynecologic oncology centers between 2012 and 2014 (Institute for Maternal and Child Health IRCCS Burlo Garofolo in Trieste and Catholic University of the Sacred Heart in Rome, Italy). We enrolled 153 patients diagnosed with clinical early (International Federation of Gynecology and Obstetrics stages I-II) type I endometrial cancer. RESULTS: Human epididymis protein 4 levels seemed to be strictly related to age (P < 0.001) and menopausal status (P < 0.002). Compared with myometrial invasion (MI), the HE4 values were significantly higher in case of invasion of greater than 50% of the thickness: MI of greater than 50%, median of 94.85 pmol/L (38.3-820.8 pmol/L), versus MI of less than 50%, median of 65.65 pmol/L (25.1-360.2 pmol/L), (P < 0.001). The HE4 levels increase significantly with increasing tumor size: diameter of larger than 2 cm, median of 86.9 pmol/L (35.8-820.8 pmol/L), versus diameter of smaller than 2 cm, median of 52.2 pmol/L (33.3-146.8 pmol/L), (P < 0.001). In our population, HE4 did not correlate with the histological grade, endometrial cancer type I versus type II (P = 0.86), the lymphovascular infiltration (P = 0.12), and the cervical invasion (P = 0.6). We established a new variable, considering 3 high-risk tumor features: MI of greater than 50% and/or histological G3 and/or type II. Human epididymis protein 4 levels significantly increase in high-risk tumors (high risk HE4, 93.6 pmol/L vs low-medium risk, 65.5 pmol/L; P < 0.001). CONCLUSIONS: A preoperative HE4 evaluation could help stratify patients with deep invasion and/or metastatic disease and is correlated with other relevant prognostic factors to be considered to tailor an adequate surgical strategy.