Lymphomas of the Vulva: A Review of the MITO Rare Cancer Group.

Since they are very rare tumors, lymphomas of the vulva are often not properly recognized. Patients with vulvar lymphoma are generally elderly and the classical manifestation of the disease is a vulvar mass. No significant age differences have been found between primary and secondary lymphoma. To make a correct diagnosis, it is therefore necessary to use not only histological examination but also the genetic and molecular profile in order to establish optimal therapeutic management. Literature analysis confirm the good prognosis of this disease.

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.

Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group.

BACKGROUND: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. METHODS: We made a systematic search of the literature, including the terms "dermatofibrosarcoma protuberans", "vulva", and "vulvar", alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. RESULTS: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. CONCLUSIONS: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients' quality of life. Management by a multidisciplinary team is highly recommended.

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.

Borderline epithelial tumors of the ovary: Experience of 55 patients.

The objective of the present study was to evaluate the clinicopathological features and the survival time estimates in patients treated for borderline ovarian tumors (BOTs). A retrospective review of all patients treated for BOTs at the University of Bari (Bari, Italy) between 1991 and 2011 was performed. Data were obtained from hospital records and gynecological oncology charts. A total of 55 patients were identified. The median age was 40 years (range, 13-79 years). The majority of the patients (85.5%) exhibited International Federation of Obstetrics and Gynecology (FIGO) stage I disease and the remainder exhibited FIGO stage II/III (7.3% in each stage). Serous histology was found in 60.0% of the cases and an elevation of the cancer antigen-125 serum level occurred in 23.6% of the cases. All patients underwent surgery and 3.7% received chemotherapy. In total, 10.9% exhibited recurrence and the median survival rate was 39 months. The median survival time and the five-year survival rate were 42 months (range, 16-84 months) and 97%, respectively. Therefore, BOTs have an excellent prognosis. Conservative surgery should be considered for patients of reproductive age who desire preservation of fertility. A long-term follow-up is highly recommended for these tumors.

Second-line chemotherapy in recurrent clear cell ovarian cancer: results from the multicenter italian trials in ovarian cancer (MITO-9).

BACKGROUND AND AIMS: Ovarian clear cell carcinoma (CCC) has a poorer prognosis than other subtypes of ovarian cancer. In this study, we evaluated the responsiveness to second-line chemotherapy in recurrent ovarian CCC. METHODS: The MITO-9 project investigated a cohort of patients observed between 1991 and 2007 in 20 centers. We identified 72 out of 240 patients with recurrent disease (28% stage I-II and 72% stage III-IV at diagnosis). RESULTS: In 56% of patients, the clear cell histology was pure. Twenty-five patients were platinum-resistant, 18 were platinum-sensitive with a platinum-free interval (PFI) of 6-12 months, and 29 had a PFI >12 months. Upon recurrence, 47% of patients were treated with platinum chemotherapy according to the PFI. The overall response rate (RR) to platinum was 80%, with 55, 100, and 80% RR in patients with PFI of 6-12, >12, and >24 months. The RR to nonplatinum agents in resistant patients was 33%. Among the nonplatinum agents used in primary and secondary resistant cases, gemcitabine, administered in 12 cases, had a higher activity (RR = 66%) compared to topotecan or liposomal doxorubicin (n = 31; RR = 33 and 10%, respectively). CONCLUSIONS: This study showed that the treatment of recurrent ovarian CCC should be based on the PFI as in the other subtypes. Data in platinum-resistant patients suggest gemcitabine as the drug with the highest activity. We recommend that gemcitabine be studied prospectively in a phase 2 trial.

Surgical and medical treatment of clear cell ovarian cancer: results from the multicenter Italian Trials in Ovarian Cancer (MITO) 9 retrospective study.

OBJECTIVE: Clear cell ovarian carcinoma has a poorer prognosis compared with other histological subtypes. MATERIALS AND METHODS: The Multicenter Italian Trials in Ovarian Cancer (MITO) 9 study retrospectively assessed an Italian cohort of patients with clear cell ovarian cancer observed in the years 1991-2007 in 20 Italian centers. RESULTS: A total of 240 patients with ovarian cancer were analyzed. Forty-five percent of the patients had stage I disease. In 62.9%, clear cell histology was pure, whereas in the other cases, a mixed population was evident. Most of the cases underwent standard surgery, whereas in 7.1% of the patients, a fertility-sparing surgery was given. Lymphadenectomy was performed in 47.9% (115/240) of the patients (54.3% in stages I and II; 39.2% in advanced stage). Most of the patients were treated with platinum-based chemotherapy including paclitaxel in 52.9%. Disease-free survival was longer in patients undergoing lymphadenectomy at surgery (P = 0.0001), both in early stages (P = 0.0258) and in stage III and IV diseases (P = 0.0037). The impact of lymphadenectomy was also evident on overall survival in patients with advanced-stage disease. At multivariate analysis, lymphadenectomy (done vs not done) and stage (I and II vs III and IV) were independently associated with longer disease-free and overall survival, whereas front-line chemotherapy (with vs without taxanes) was not significant. CONCLUSION: This analysis suggests that lymphadenectomy has a strong prognostic role for clear cell ovarian cancer influencing disease-free survival and overall survival. The addition of paclitaxel to platinum-based chemotherapy does not affect the outcome.