Effectiveness and tolerance of electrochemotherapy as palliative therapy for patients with head and neck cancer and malignant melanoma and its relation to early skin reaction

Abstract Objectives To evaluate the efficacy and tolerance after the electrochemotherapy treatment for local therapy of cutaneous and subcutaneous metastases of head-and-neck tumors and malignant melanoma refractory to standard therapies, mainly in neck metastasis of squamous cell carcinoma. And, to evaluate the relation of this response according to the skin reaction (healing with ulcer or dry crust). Methods prospective pase II, observational clinical study of 56 patients with metastases of head-and-neck squamous cell carcinoma (n = 13), papillary thyroid carcinoma (n = 4), adenoid cystic carcinoma of parotid gland (n = 1) or malignant melanoma (n = 37, 5 in head). Patients were treated by electrochemotherapy (application of electrical pulses into the tumor) after the administration of a single intravenous dose of bleomycin. Kaplan-Meier curves were performed. The statistical significance was evaluated using log-rank test; p-value of less than 0.05 was considered as significant. Results Overall clinical response was observed in 47 patients (84%). Local side effects were mild in all the patients. Ten patients (76.9%) with neck metastasis of squamous cell carcinoma had some degree of response, but only in one was complete. Patients even with only partial response had a higher overall survival than patients without response (p = 0.02). Most of the patients with squamous cell carcinoma had diminution of pain and anxiety. Response rate and overall survival was higher in MM patients (86.5%) than in squamous cell cancer patients (76.9%) (p = 0.043). The healing process (dry crust/ulcer) was not associated with the overall survival (p = 0.86). Conclusions Electrochemotherapy is associated a higher overall survival and diminution of pain and anxiety. Therefore, it is an option as palliative treatment for patients with neck metastasis of squamous cell carcinoma refractory to other therapies or even as a concomitant treatment with newer immunotherapies. The type of healing of the surgical wound could not be associated with a higher rate of response or survival. Level of evidence III.

Primary Anorectal Melanoma: Case Report and Review of a Rare Neoplasm

Primary anorectal melanoma is a rare malignant melanocytic neoplasm; its principal manifestation is rectal bleeding. It has an ominous prognosis with a five-year survival rate of 10%. The case of a 56-year-old woman with rectal bleeding and the sensation of a rectal mass is presented. A polypoid lesion, resected transanally, was documented in the distal rectum during the colonoscopy. The histological study confirmed a primary anorectal melanoma.

El melanoma anorrectal primario es una neoplasia melanocítica maligna poco frecuente, su principal manifestación es el sangrado rectal. Tiene un pronóstico ominoso con una tasa de sobrevida del 10% a 5 años. Se presenta el caso de una mujer de 56 años con rectorragia y sensación de masa rectal. Durante la colonoscopia se documentó una lesión polipoide en el recto distal, que se resecó por vía transanal. El estudio histológico confirmó la presencia de un melanoma anorrectal primario.

Many faces of malignant melanoma: Histopathological evaluation of liver biopsies

Background: Malignant melanoma (MM) is an aggressive tumor characterized by high metastatical potential and is sometimes diagnosed by distant organ metastasis such as liver. Morphologically it may mimic many other tumors and cause diagnostic challenges. In this paper, eleven MM cases metastasized to the liver with different morphologic patterns were analyzed in the light of literature. Materials and Methods: Histopathological and immunohistochemical findings of the cases diagnosed as MM metastasis in liver were reevaluated in the light of clinical data. Results: We obtained 11 MM cases with hepatic metastasis. In slides of the first four cases, morphologic features similar to epithelioid variant of MM with different amounts of melanin deposition were observed. In the fifth and sixth cases, fascicular patterned tumoral lesion composed of spindled cells were detected; and in the seventh and eighth cases, nested and sheet-like patterned tumoral lesion consist of giant bizarre and spindled cells with hyperchromatic nuclei were observed. The last three cases consisted of plasmacytoid cells with eccentric nuclei in pseudoalveolar pattern; and the exact diagnosis was possible with the help of immunohistochemistry. Conclusion: MM should always be kept in mind in differential diagnosis of metastatic hepatic tumors with unknown primary because of its various morphological characteristics.

Malignant Melanoma Infiltrating the Bone Marrow.

Malignant melanoma with infiltration into the bone marrow is seldom reported in the literature, for they are exceedingly rare. The primary site is not always apparent and a sizeable number of cases have been attributed to an occult primary. Metastasis to bone marrow is a terminal event usually occurring in stage IV of the disease and can be a focus of residual tumor cells which can cause a relapse.The current documentation is of a case of melanoma occurring as a rectal primary with anemia, thrombocytopenia, and leukoerythroblastic reaction. The marrow aspirates and trephine biopsy showed round to spindle-shaped malignant cells with intracytoplasmic brown-black coarse pigment, suggestive of melanin. The patient was diagnosed with stage IV melanoma but was lost for follow-up. The recognition of such an entity is important for both pathologists and clinicians alike. This case is being reported for the novelty of such an occurrence.

Primary malignant melanoma with less pigmentations of urinary bladder: a case report / 中华泌尿外科杂志

An 85-year-old woman with gross hematuria for 10 days was presented to Wuxi No.2 People′s Hospital on Dec 10th, 2018.The images showed bladder mass. Pathological analysis of transurethrally resected tissue showed primary malignant melanoma with less pigmentations of urinary bladder. Without any following treatment, it grew again after 17 months. After the second surgery, she died of cachexia half a month later. Primary malignant melanoma with less pigmentations of urinary bladder is rare. It is vulnerable to misdiagnosis without immunohistochemical staining. The prognosis is poor.

Primary Malignant Melanoma of the esophagus in a 39-year-old Filipino male: A case report

@#Primary malignant melanoma of the esophagus (PMME) is an exceptionally rare condition, representing a mere 0.1 to 0.2% of esophageal cancers, and accounting for just 0.1 to 0.5% of all melanomas. This case involves a 39 -year-old Filipino male who sought medical attention after an episode of choking. Subsequently, endoscopy with biopsy revealed a mass in the distal third of the esophagus, ultimately diagnosed as PMME based on histopathology and immunohistochemistry. FDG-PET/CT scan revealed a hypermetabolic distal esophageal mass and a confluent upper paratracheal lymphadenopathy. He was initially treated with Pembrolizumab, Nivolumab, and Ipilimumab immunotherapy. However, post-treatment FDG PET/CT scans unveiled metabolic progression of the esophageal mass with new hypermetabolic cervical lymph nodes, necessitating a shift to carboplatin and paclitaxel chemotherapy. After two cycles, there was a notable metabolic regression of the mass and paratracheal node with metabolic resolution of the cervical lymph node. An additional 2 cycles of chemotherapy were given, aimed to further reduce the size of the tumor, however, a succeeding follow-up study revealed metabolic progression of the mass. Surgical resection of both the esophageal mass and paratracheal nodes became imperative. The aggressive characteristics, metastasis at early diagnosis, and lack of effective treatment have contributed to the poor prognosis of PMME. Total esophagectomy is the preferred method of treatment. Chemotherapy and immunotherapy may be used in advanced diseases but with variable efficacy. The utilization of FDG PET/CT scans plays a crucial role in both the initial staging and the ongoing assessment of treatment response in patients diagnosed with PMME. This advanced imaging modality offers valuable insights into the extent of the disease and aids clinicians in evaluating the effectiveness of the chosen therapeutic interventions. Given the rarity and challenges associated with PMME, a multidisciplinary approach integrating surgical, medical, and imaging strategies is essential for comprehensive patient care.

Melanoma maligno cutáneo con metástasis sistémica en una hembra de tigre de bengala blanca (Panthera tigris tigris) / Cutaneous Malignant Melanoma with Systemic Metastasis in a Female White Bengal Tiger (Panthera tigris tigris)

Resumen La presencia del melanoma maligno cutáneo en felinos exóticos está pobremente reportada. Una hembra de tigre de Bengala blanca (Panthera tigris tigris), de 6 años, no castrada, solitaria y viviendo en un ambiente adecuado, fue evaluada por la presencia de una masa cerca del ojo izquierdo. La histopatología de la biopsia remitida mostró la presencia de melanocitos con características anaplásicas (pleomorfismo, citomegalia, cariomegalia, binucleación), área hemorrágica y foco inflamatorio fibrinoso. El diagnóstico histopatológico fue melanoma maligno. Dos meses después del diagnóstico, la tigresa manifestó un deterioro considerable con pérdida de peso y presencia de una masa redonda en el cuello. En aquel momento, por su tamaño, el melanoma obstruía totalmente la visión. Con base en esto, y por razones humanitarias, el animal fue eutanasiado. Al momento de la necropsia, se observó metástasis en pulmones, pleura y riñones. Este caso evidencia la importancia de dar a conocer la presentación de melanomas en especies poco reportadas.

Abstract The occurrence of cutaneous malignant melanoma in exotic felids is poorly reported. A 6-year-old, unneutered, solitary, female white Bengal tiger (Panthera tigris tigris), living in a suitable environment, was evaluated for the presence of a mass near the left eye. Histopathology of the referred biopsy showed the presence of melanocytes with anaplastic features (pleomorphism, cytomegaly, karyomegaly, binucleation), hemorrhagic area and fibrinous inflammatory focus. The histopathological diagnosis was malignant melanoma. Two months after diagnosis, the tigress manifested considerable deterioration with weight loss and the presence of a round mass on the neck. At that time, because of its size, the melanoma totally obstructed vision. Based on this, and for humane reasons, the animal was euthanized. At the time of necropsy, metastasis was observed in the lungs, pleura and kidneys. This case demonstrates the importance of raising awareness of melanomas in underreported species.

Melanoma maligno primario de pulmón / Primary malignant lung melanoma

Introducción: El melanoma maligno es un tumor de etiología multifactorial, cuando afecta al sistema respiratorio su origen es casi siempre metastásico, y como tumor primario es muy infrecuente, constituye el 0,07 por ciento de los tumores pulmonares malignos. Objetivo: Presentar un caso clínico con diagnóstico de melanoma maligno primario de pulmón. Presentación del caso: Se presenta el caso de una mujer de 64 años de edad, exfumadora, sin antecedentes de melanoma, que presenta tos seca asociada a disnea, los esfuerzos físicos y dolor torácico. Además de astenia y pérdida de peso. Se le realizó biopsia pulmonar mediante broncoscopia y se le diagnosticó melanoma. Conclusiones: El melanoma primario pulmonar es uno de los más raros del tipo de melanoma visceral, es una entidad neumológica infrecuente y el diagnóstico preciso requiere una investigación detallada y el cumplimiento de criterios clínicos y anatomopatológicos específicos(AU)

Introduction: Malignant melanoma is a tumor of multifactorial etiology, when it affects the respiratory system its origin is almost always metastatic, and as a primary tumor it is very infrequent, it constitutes 0.07 por ciento of malignant lung tumors. Objective: To report a clinical case with a diagnosis of primary malignant melanoma of the lung. Case report: We report the case of a 64-year-old woman, former smoker, with no history of melanoma, who had dry cough associated with dyspnea with physical exertion and chest pain. In addition to asthenia and weight loss, she had a lung biopsy by bronchoscopy and was diagnosed with melanoma. Conclusions: Primary pulmonary melanoma is one of the rarest of the visceral melanoma type, this is an infrequent pneumological entity and the precise diagnosis requires detailed investigation and the fulfillment of specific clinical and pathological criteria(AU)

Melanoma amelanótico diseminado / Disseminated amelanotic melanoma

El melanoma amelanótico es una de las neoplasias con mayor índice de mortalidad por su alta agresividad y baja probabilidad diagnóstica. Afecta a la población de todo el orbe, más frecuente en caucásicos, con predisposición genética y factores de riesgo como la exposición al sol. Presenta tasas de supervivencia menor a 10 por ciento a 5 años y de recurrencia elevadas; con evidencia de procesos metastásicos a distancia en órganos como cerebro, tejido celular subcutáneo, pulmón, peritoneo, hueso, lo que ensombrece el pronóstico. Se presenta el caso de una paciente de 21 años de edad que acude al hospital por presentar lesiones equimóticas, nódulos subcutáneos y cefalea hemicránea izquierda de dos meses de evolución. Se le realizó tomografías de tórax abdomen y resonancia magnética de cráneo y evidenciaron diseminación metastásica. Se realizó estudio histopatológico con inmunohistoquímica que informó melanoma amelanótico(AU)

Amelanotic melanoma is one of the neoplasms with the highest mortality rate because it is highly aggressive and the diagnostic probability is low. It affects the population of the entire globe, more frequent in Caucasians, with genetic predisposition and risk factors such as sun exposure. It presents survival rates of less than 10 percent at 5 years and high recurrence rates; with evidence of distant metastatic processes in organs such as brain, subcutaneous cellular tissue, lung, peritoneum, bone, which casts a shadow on the prognosis. We report the case of a 21-year-old patient who came to the hospital due to ecchymotic lesions, subcutaneous nodules and a two-month evolution of left hemicrania headache. She underwent chest and abdomen tomography and MRI of the skull. They showed metastatic spread. Histopathological study was performed with immunohistochemistry that reported amelanotic melanoma(AU)

Extensive malignant melanoma of the oral cavity: a rare occurrence

Primary malignant melanoma of the oral cavity is a rare tumor in clinical practice. Extensive malignant melanomas are still very rare in the literature. Patients with malignant melanoma of oral cavity are often diagnosed at the advanced stage of the disease due to their painless and nonspecific radiological findings. Histopathology is the gold standard for the final diagnosis and staging of the tumor. Surgery followed by radiotherapy is the standard treatment offered to patients with malignant melanoma. Here we present a rare case of extensive malignant melanoma of oral cavity which was successfully managed by surgical excision followed by adjuvant radiotherapy.

In vitro and in vivo evaluation of folate-mediated triptolide exosome targeted delivery system against malignant melanoma / 药学学报

In this study, exosomes were extracted from human malignant melanoma cell A375. Folic acid (FA) receptor was used as target and triptolide (TPL) was used as model drug to prepare exosome targeted drug delivery system, FA-Exo/TPL. The physicochemical properties and antitumor effect were evaluated in vivo and in vitro. Gradient centrifugation method was applied to collect exosomes. Then, exosome was modified with FA for loading TPL. The particle sizes of the FA-Exo/TPL were about 100 nm with a double-layer membrane structure like a tray. It is characteristic of high encapsulation efficiency and drug loading. In vitro experiments showed that FA-Exo/TPL could be effectively uptaken by A375 cells, thus significantly inhibiting proliferation and promoting apoptosis the cells. In vivo experiment results showed that FA-Exo/TPL could effectively inhibit the growth of tumor tissue, prolong the model mice life cycle, and significantly reduce the systemic toxicity of the free drug, playing a synergistic and toxic role. Animal welfare and experimental procedures follow the regulations of the Animal Ethics Committee of Fudan University Shanghai Cancer Center. This study provides a new strategies and methods for the preparation of TPL against malignant melanoma.

Primary malignant melanoma of the lung: a case report and literature review / 中国医师杂志

Objective:To analyze and summarize the clinical features, diagnosis and treatment of primary malignant melanoma of the lung (PMML).Methods:To report a confirmed case. And searched the keywords " malignant melanoma, primary, lung" from the databases of CNKI, Weipu, Wanfang and PubMed, then reviewed the relevant literature.Results:A 65-year-old male admitted to the First Affiliated Hospital of Zhengzhou University with the complaint of frequent coughs with bloody sputum for 2 months. Computed tomography (CT) showed a large space-occupying lesion in the upper lobe of the right lung and to be a hyper metabolic tumor by positron emission tomography-CT. The pathological biopsy of the mass confirmed a malignant tumor cell, immunohistochemical results showed S-100(+ ), SOX-10(+ ), HMB45(+ ), MelanA(-), AE1/AE3(-), CK5/6(-), Ki-67(40%). The patient died after 3 months of diagnosis because of refusing any further therapy. There were 36 papers searched from mentioned databases reported 41 Chinese patients with PMML. And we analyzed the clinical data of 42 cases (include the present case) and discussed the diagnosis and treatment while referring to the existing literatures.Conclusions:PMML is extremely rare, and difficult to differentiate from lung cancer, A diagnosis of PMML is based on the combination of clinical, imaging characteristics and pathological outcomes. The choice of treatment is an aggressive surgical approach, combined with radiation therapy and chemotherapy. Because of high degree of malignancy, powerful capacity for invasiveness and recurrence, the overall prognosis is poor.

Effect of piceatannol against malignant melanoma / 华西口腔医学杂志

OBJECTIVES@#To study the antitumor effect of piceatannol (PIC) on malignant melanoma @*METHODS@#B16F10 cells were cultured @*RESULTS@#The cell viability of B16F10 decreased with increasing PIC concentration. The results of the Transwell assay showed that invasion ability decreased with increasing PIC concentration, and healing time was prolonged at increased PIC concentration in the wound healing assay. Western blot results showed that PIC mainly inhibited the phosphorylation of Syk and inhibited the expression of MMP-2, MMP-9, and VEGF. RNA interference pointed out that blocking the expression of Syk can reveal the same inhibition effect on B16F10 cells as PIC. @*CONCLUSIONS@#PIC might block the progression of malignant melanoma by inhibiting spleen tyrosine kinase.

Clinical analysis of 19 cases of oral mucosal malignant melanoma / 口腔疾病防治

Objective@#To investigate the clinicopathological features, treatment and prognosis of oralmucosal malignant melanoma to provide a reference for clinical practice.@*Methods@#Data from 19 patients with oralmucosal malignant melanoma were collected, and their clinical manifestations, treatment methods and follow-up results were retrospectively analyzed. @*Results@#Among the 19 patients, 11 cases (58%) had lesions in the gingiva, 7 cases (37%) had lesions in the palate, and 1 case (5%) had lesions in the tongue, the difference was statistically significant (P<0.05). Eight patients had regional lymph node metastasis with a metastasis rate of 42%, of which 4 cases had multiple site metastasis, and the total number of regional lymph node metastasis sites was 15. Among the 19 patients, 3 cases received only surgery, 4 cases received cryotherapy, and 12 cases received combined surgery, cryotherapy and biological immunotherapy. Pathological examination showed malignant melanoma. The positive rates of S-100, HMB-45 and Melan-A were 95%, 89% and 84%, respectively. Kaplan-Meier survival analysis showed that patients with lesions less than 5 cm2 had a higher survival rate (P < 0.05).@*Conclusions@# Oral malignant melanomas usually present as black lesions in the oral mucosa, which are prone to metastasis in early stage. The area of lesions may affect the prognosis of the disease. Therefore, the large range of black lesions or masses should be the alert for the clinicians. Oral malignant melanoma patients are usually treated with combined treatment with surgery, cryotherapy and biological immunotherapy.

Primary malignant melanoma of vagina: a case report

Primary malignant melanoma of the vagina is a rare and aggressive disease with worse prognosis as compared with non-genital melanomas or other vaginal malignant neoplasms. Presented here is a case of 42 years female with 3 months history of amenorrhea and vaginal discharge. On vaginal examination, a firm growth of size approximately 7-8 cm was found attached to the left postero-lateral wall of vagina and extending up to the introitus. On biopsy and histopathological examination, it was diagnosed as a case of high-grade malignant melanoma of amelanotic type. Radiotherapy was started as a part of treatment after consultation with an oncologist, considering non-resectable nature of the mass. The patient received first 5 cycles of radiotherapy but succumbed to the disease during treatment.

Transcatheter arterial infusion of anti-programmed cell death 1 antibody pembrolizumab combined with temozolomide or nab-paclitaxel in patient with primary anorectal malignant melanoma: Four case reports

Primary anorectal malignant melanoma (ARMM) is an extremely rare but aggressive tumor. We assessed the efficacy and safety of transcatheter arterial infusion (TAI) with anti-PD-1 antibody pembrolizumab at a dosage of 100 mg with 0.9% NaCl at a volume of 100 mL administered over a 30-min period every 3 weeks, combined with temozolomide or albumin-bound paclitaxel (nab-paclitaxel) in four patients with ARMM. Temozolomide was administered orally once per day at a dosage of 200 mg/m2/d for five consecutive days about every 4 weeks. Nab-paclitaxel was administered at a dosage of 200mg/m2/d once about every 3 weeks. Among four patients with a median follow-up of 8.9 months, two cases showed Murine Double Minute 2 (MDM2) amplification. Case 1 with Stage II ARMM showed pathological complete response after four cycles of TAI with pembrolizumab combined with nab-paclitaxel. Case 4 was at Stage II and showed stable disease consistently throughout the treatment. Case 2 was at stage II and Case 3 was at stage III, and they showed partial response after four or three cycles, respectively, of TAI with pembrolizumab combined with temozolomide. No Grades 3–4 adverse reactions were observed. Therefore, a combination of TAI with pembrolizumab and temozolomide or with nab-paclitaxel appears to be a promising option for treating ARMM. However, multicenter clinical trials are required to confirm the efficacy and safety of this procedure

Coexistence of anal malignant melanoma and papillary renal cell cancer: An interesting coexistence

Coexistence of malignant melanoma and renal cell cancer (RCC) is a rare phenomenon, but this issue becomes increasingly popular. The objective of the current study is to present a case with coexistent anorectal melanoma (ANM) and papillary RCC detected. A 61-year-old female admitted to our clinic with complaints of blood in the stool. ANM diagnosed with colonoscopic biopsy and a mass lession with a size of 57 mm × 53 mm suggesting RCC was detected in the left kidney during staging procedure. Transabdominal resection and radical nephrectomy were performed and diagnoses of ANM and papillary RCC were confirmed. Adjuvant radiotherapy was applied for ANM. The patient is still under follow-up for 6 months and no recurrence or progression was detected. To the best of our knowledge, this is the first report of this interesting coexistency

Malignant Melanoma of Pleura: A Case Report and Literature Review / 中国肺癌杂志

Malignant melanoma is a kind of tumor produced by human melanocytes. It has a high degree of malignancy, early metastasis and high mortality. Most melanomas are caused by malignant skin nevus and can also be seen in the digestive tract such as rectum and anus. Primary malignant melanoma of pleura is rare, rarely seen in case reports. This paper reports the clinical data of a case of malignant melanoma with cough, expectoration and pleural effusion as the first symptoms diagnosed by thoracoscopy combined with pathology in Beijing Friendship Hospital Affiliated to Capital Medical University, and analyzes and summarizes the literature data.

The application value of transrectal ultrasonography in the diagnosis of anorectal malignant melanoma / 中华超声影像学杂志

Objective@#To investigate the value of transrectal ultrasonography(TRUS) in the preoperative diagnosis and tumor T-staging of anorectal malignant melanoma(ARMM).@*Methods@#The clinical and ultrasound data of 19 patients(22 lesions) with ARMM confirmed by pathology from February 2008 to Apirl 2019 in Fujian Medical University Affiliated Union Hospital were collected. TRUS was performed within one week before pathological examination. The sonographic features were summarized, ultrasonic typing was performed according to the lesion size and growth pattern, and the accuracies of preoperative ultrasound diagnosis and tumor staging were analyzed.@*Results@#Among the 22 lesions, 63.64%(14/22) were with a distance of ≤3 cm between the anal verge and the lower margin of tumor, 68.18%(15/22) with regular shape, 81.82%(18/22) with clear boundary, and 63.64%(14/22) with Adler blood flow grading from Ⅱ to Ⅲ. The preoperative TRUS diagnostic coincidence rate was 73.68%(14/19), and the tumor T-staging accuracy was 75%(12/16), respectively.@*Conclusions@#TRUS exhibits certain sonographic characteristics, with high preoperative diagnosis rate and tumor T-staging accuracy in anorectal malignant melanoma, which is of great guiding significance for clinical diagnosis and treatment.

Primary anorectal malignant melanoma: A clinical, radiology and pathology correlation

@#Introduction: Primary gastrointestinal melanomas are mucosal malignancies that arise from melanocytes in the oropharynx, rectum, and anus. Anorectal malignant melanoma (ARMM) are exceedingly rare, accounting for less than 1% of all melanomas, 0.1% of all rectal malignancies and 4% of anal malignancies. Diagnosis is frequently delayed as these lesions are often mistaken for haemorrhoids. Histological evaluation with special immunohistochemical stains is often necessary for definitive diagnosis. Due to the aggressive nature, 61% of patients with ARMM would already have lymph node involvement or distant metastases, by the time of diagnosis. Prognosis is usually poor with 5-year survival rate of <20%. We report a case of metastatic ARMM in an elderly lady who presented with symptoms and signs mimicking a haemorrhoid. Case Report: A 69-year-old lady presented with one year history of intermittent rectal bleed and an anorectal mass that was initially treated as haemorrhoid. Colonoscopy showed a hyperpigmented mass in the anorectal region which was confirmed as malignant melanoma on histopathological examination. Imaging with CT and MRI demonstrated locally advanced tumour with distant metastases to the liver and lung. Patient was referred for palliative management. Conclusion: ARMM is a rare malignancy and often presented with non-specific clinical signs. Diagnosis is frequently delayed without high index of suspicion. MRI pelvis is the imaging of choice to assess local extent of disease. Histologic evaluation with special immunohistochemical stains is often necessary for definitive diagnosis. Prognosis is poor despite surgical and chemotherapeutic interventions.