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Hindawi Case Reports in Oncological Medicine Volume 2019, Article ID 1018492, 4 pages https://doi.org/10.1155/2019/1018492 Case Report A Case of Stage I Vulvar Squamous Cell Carcinoma with Early Relapse and Rapid Disease Progression 1 1 1 2 1 Marta Peri, Antonino Grassadonia , Laura Iezzi, Patrizia Vici, Michele De Tursi, 1 1 3 Clara Natoli , Nicola Tinari, and Marinella Zilli Medical Oncology Unit, Department of Medical, Oral and Biotechnological Sciences, University “G. D’Annunzio”, Chieti, Italy Division of Medical Oncology 2, IRCCS Regina Elena National Cancer Institute, Rome, Italy Medical Oncology Unit, SS Annunziata Hospital, Chieti, Italy Correspondence should be addressed to Antonino Grassadonia; firstname.lastname@example.org Received 6 March 2019; Accepted 23 June 2019; Published 3 July 2019 Academic Editor: Jose I. Mayordomo Copyright © 2019 Marta Peri et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Squamous cell carcinoma (SCC) is the most common subtype of vulvar cancer. Locoregional surgery is often curative when the tumor is diagnosed at an early stage. However, the disease can unexpectedly evolve with a dismal prognosis even after an early diagnosis. We report a case of a woman who experienced a rapid, chemorefractory tumor progression after surgery for stage IB vulvar SCC. 1. Introduction The dissemination pattern of vulvar carcinoma is mostly lymphogenic, and the inguinofemoral lymph nodes are the Vulvar cancer is the fourth most common gynecologic cancer primary site of regional spread . Distant spread usually after endometrial, ovarian, and cervical cancer, accounting occurs late in the course of the disease. In the absence of for about 5% of all female genital tract malignancies . distant metastases, the most important prognostic factor is The most common histological type of vulvar cancer is represented by pathologic status of the inguinal nodes, squamous cell carcinoma (SCC), which accounts for about while the size of the primary tumor is less important in 90% of the cases. Two diﬀerent types of SCC have been deﬁning prognosis . described: a keratinizing form and a warty/basaloid form Herein, we describe a case of vulvar carcinoma diagnosed . The former occurs predominantly in postmenopausal at stage IB FIGO that displayed a very aggressive behavior. women with a background of lichen sclerosus or lichen pla- The patient experienced early local recurrence and rapid nus evolving in diﬀerentiated vulvar intraepithelial neoplasia metastatic disease progression causing death just a few (d-VIN) and is associated with poorer prognosis [3, 4]. The months after relapse. latter is more common in younger patients and is related to high-risk papillomavirus (HPV) infection evolving in high- 2. Case Presentation grade squamous intraepithelial lesion (HSIL or VIN3) [3, 5]. Radical local excision with inguinofemoral lymph node A 70-year-old woman presented at the gynecology unit of dissection currently represents the standard of treatment our hospital complaining about a painful vulvar lesion in for women with vulvar cancer . Since surgery is associated May 2017. She had no signiﬁcant medical history. Physical with high morbidity, noninvasive methods are commonly examination revealed an exophytic and ulcerative vulvar utilized to evaluate the extension of disease in the preopera- mass, approximately 4 cm in diameter, localized on the right tive setting. In particular, magnetic resonance imaging labium majus at less than 2 cm from the midline, without pal- (MRI) is helpful for the assessment of local tumor extension pable inguinal lymph nodes bilaterally. An incisional biopsy and inguinal lymph node involvement . was performed, and histology revealed an invasive poorly 2 Case Reports in Oncological Medicine (a) (b) Figure 1: Cutaneous tumor progression during the ﬁrst-line chemotherapy. Erythematosus nodules on the skin of the right groin (a) and thigh (b). diﬀerentiated vulvar SCC. A total-body CT scan performed to stage the disease resulted negative for distant metastases. The patient underwent right hemivulvectomy in order to obtain wide tumor-free pathological margins in June 2017. Concomitant inguinal lymph node dissection was not per- formed due to the patient’s refusal (risk of lymphedema). Histopathologic ﬁndings conﬁrmed a poorly diﬀerentiated vulvar SCC arising on a background of lichen sclerosus. The size of the invasive SCC lesion was 4.5 cm with a depth of invasion of 2.7 mm and no lymphovascular invasion. All surgical margins of the lesion were tumor-free (more than 1 cm). She was addressed to our oncology unit in July 2017. We required a disease restaging by abdominal and pelvic MRI scan and chest CT scan. No evidence of distant metastases resulted from the imaging studies. Therefore, we suggested locoregional lymph node dissection in order to deﬁne the pathologic stage of the tumor and to plan postoperative adju- vant radiotherapy to the groin just in case of lymph node involvement. In August 2017, a bilateral inguinofemoral lymph node dissection was performed with all nodes (twelve) resulting Figure 2: Further cutaneous progression during the second-line chemotherapy. Ulceration and ﬁstulization of the right groin negative for metastatic spread on conventional hematoxylin- nodule. eosin staining. The tumor was staged as FIGO stage IB, and the patient was addressed to strict follow-up. However, just one month later (September 2017), the Because of recurrence, systemic chemotherapy was patient developed a local recurrence with a 3 cm nodule started with carboplatin (AUC5 day 1 every 3 weeks) and in the right vulvar area and a 0.8 cm lesion in the clitoris. paclitaxel (80 mg/m days 1 and 8 every 3 weeks). After 3 A wide local excision was performed and histopathology cycles, a total body CT scan showed progression of metastatic examination revealed a poorly diﬀerentiated vulvar SCC disease in the lungs, lymph nodes, and liver. Moreover, a in both lesions. painful erythematosus nodule appeared on the skin of the A restaging CT scan of the chest, abdomen, and pelvis right groin (Figure 1(a)) and right thigh (Figure 1(b)). showed multiple bilateral pulmonary metastases and multi- Because of disease progression, a second-line chemo- ple inguinal and pelvic lymph node involvement. therapy with capecitabine (1000 mg/m bid, days 1-14 every Case Reports in Oncological Medicine 3 (a) (b) Figure 3: Further systemic tumor progression during the second-line chemotherapy. CT scan showing multiple lesions in the lungs (a), some excavated (arrow), and matted metastatic lymph nodes in the iliac/inguinal region (b) (arrows). 21 days) was started (December 2017). After 3 cycles of in the case herein described, could play a pivotal role in deter- treatment, the patient presented ulceration and ﬁstuliza- mining a poor prognosis [4, 10]. tion of the groin lesion (Figure 2) and new skin nodules Hematogenous spread to distant sites, including the lung, in the right thigh associated with extremities lymphedema. liver, and bone, usually occurs late in the natural history of She complained of perineal pain and analgesic therapy was the disease. Cutaneous metastases from vulvar carcinoma prescribed. Moreover, palliative radiotherapy to inguinal have been rarely described, but when documented, they are metastases (30 Gy in 10 fractions) was performed. associated with short survival [11–15]. The median time to A reevaluation CT scan (February 2018) revealed further death from the diagnosis of skin metastasis has been esti- progression of the disease with multiple liver metastases, mated to be around 6 months [4, 15]. multiple excavated lesions in the lungs (Figure 3(a)), and In our case, distant metastases developed shortly after matted metastatic iliac/inguinal lymph nodes (Figure 3(b)). diagnosis, in an early inﬁltrating phase of the disease (only The patient died one month later, in March 2018, because 2.7 mm). Skin metastases also manifested early during the of respiratory failure. metastatic phase, in the course of the ﬁrst-line systemic treatment. Consistent with literature data, the appearance of cutaneous metastases was a predictor of short survival 3. Discussion for our patient. She died after only 4 months. The rapid tumor progression was accompanied by a We described a case of vulvar SCC that, despite an early- chemoresistant phenotype with a dismal prognosis. In fact, stage presentation at diagnosis, rapidly evolved into meta- disease progressed during the ﬁrst-line platinum-based static chemoresistant disease, leading the patient to death chemotherapy in all the secondary sites and further pro- in a few months. gressed during the subsequent second-line treatment with Vulvar SCC is a rare disease mainly occurring in postmen- capecitabine. opausal women. The prognosis for patients with early-stage To date, the therapeutic management of metastatic disease is generally good, but cancer can spread from its orig- vulvar SCC is heterogeneous and data are insuﬃcient to recommend a preferred chemotherapeutic regimen in the inal site to locoregional nodes and/or distant organs by lym- phatic embolization or hematogenous diﬀusion, respectively. palliative setting. Usually, regimens with proved eﬃcacy in Lymphatic spread represents an early event in the course of advanced cervical or anal cancers are chosen for the treat- the disease and involves ipsilateral inguinal, femoral, and pel- ment of metastatic vulvar SCC . vic lymph nodes, usually in a sequential manner. Lymph node Advances in the molecular biology of vulvar SCC may provide insight into the future management of this tumor status remains the single most important prognostic factor. The 5-year overall survival is more than 90% for patients . At the moment, despite the two distinct etiologies, there without nodal involvement, which reduces to less than 60% is no speciﬁc recommended chemotherapeutic regimen for in the case of nodal involvement . HPV-associated or HPV-independent tumors. Our patient presented with a negative pathological nodal status at diagnosis, but unfortunately, her disease-free sur- 4. Conclusion vival was very short, with local relapse occurring just one month after surgery. It is evident that other prognostic fac- Vulvar SCC can unexpectedly evolve with a dismal prognosis tors other than stage may inﬂuence the natural history of even if diagnosed at an early stage. The disease can be rapidly the disease. For example, tumor characteristics such as progressive and refractory to chemotherapy. Novel therapeu- high-grade or lichen sclerosus-related etiopathogenesis, as tic approaches are required. 4 Case Reports in Oncological Medicine vulvar squamous cell carcinoma,” International Journal of Conflicts of Interest Gynecological Cancer, vol. 14, no. 2, pp. 384–387, 2004. The authors declare that they have no conﬂicts of interest.  A. R. Wang, M. O'Brien, R. Ross, T. Long, and L. 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