Proximal epithelioid sarcoma of the vulva: A case report and review of the literature

Primary sarcomas of the vulva are rare, with an incidence of 1.5% to 5% of all vulvar malignancies. There are only 41 cases described in the English literature of proximal epithelioid sarcoma of the vulva, which also constitutes a highly aggressive neoplasm with a high rate of recurrence and metastasis. We are reporting the first case of vulvar epithelioid sarcoma reported in our country, Peru, and Latin America. We present the case of a 53-year-old woman with no significant history who presented a large tumor mass of 25 cm in the left hemivulva of 2 years of evolution, associated with intense pain. She underwent radical surgical resection with left inguinopelvic lymphadenectomy and reconstruction of Rectus Abdominal Muscle Myocutaneous Flaps (TRAM).

Cranial and Spinal Malignant Peripheral Nerve Sheath Tumor: A Pathological Enigma

Objective Malignant peripheral nerve sheath tumor (MPNST) arises from nerve sheaths, mostly seen in peripheral nerves but rare in craniospinal nerves. The information available in the literature to build up treatment strategy and improve clinical outcomes is scarce. We are reviewing cases from our institute, with emphasis on radiological features for early differentiation from its benign variants. Methods We analyzed pathologically diagnosed cases retrospectively from January 2007 to December 2018 at our institute. Clinicoradiological details and treatment parameters were collected from medical records for evaluation. Each case was contacted telephonically for final clinical follow-up at the time of writing the manuscript. Results A total of seven cases of MPNST were diagnosed in the last 10 years. It included four intracranial and three spinal cases. The mean age for the cohort was 34.3 years, with five females. We could achieve gross total resection (GTR) and subtotal resection in four (57.1%) and two (28.6%) cases, respectively. We could achieve an overall survival of 57.1% in the average follow-up of 28.2 months (range: 8–84 months). Conclusion MPNST is a rare tumor with a bad prognosis. Radical surgical resection is the mainstay of the treatment, but it is not always possible to achieve it because of the inaccessible location and large size of lesions. Preoperative diagnosis is challenging; however, few radiological findings may give a clue toward it. As a disease entity overall, it has a poor outcome with a high rate of fatality.

Primary cardiac angiosarcoma: a case report

Primary cardiac angiosarcoma is relatively rare, and most cases involve metastasis at the time of diagnosis. The median survival time is 14 months for patients who can be treated surgically, versus 3.8 ± 2.5 months for patients with metastasis who could not undergo surgery. Radical surgical resection, radiotherapy, chemotherapy, and targeted therapy are the main treatments, but prognosis remains poor because of rapid progression and high recurrence and metastasis rates. At present, there is no unified standard treatment, and selecting the correct treatment plan and improving patient survival and quality of life remain challenging. We have reported the case of a 45-year-old woman with a primary heart tumor that infiltrated the right atrial wall and pericardium. Angiosarcoma was verified histologically. After palliative resection of the primary tumor followed by concurrent chemoradiotherapy and targeted therapy, the patient exhibited overall survival of 23 months, highlighting the potential utility of this treatment strategy.

Colloid Carcinoma of the Pancreas: A Case Report

Background: Pancreatic colloid carcinoma is a rare pancreatic cancer, which is a subtype of pancreatic ductal adenocarcinoma. Case: a 71-year-old woman with a tumor of about 1.9x1.3cm in size located in the neck and body of the pancreas without invasion of surrounding organs. The patient underwent body and tail pancreatectomy, and the pathology revealed an intraductal papillary mucinous tumor of the pancreas with associated infiltrating colloid carcinoma. The patient recovered well after surgical treatment. Conclusion: Pancreatic colloid carcinoma is a malignant tumor, but it has a lower degree of malignancy and a better prognosis compared with pancreatic ductal adenocarcinoma. Currently, radical surgical resection is the main treatment principle.

Results of surgical treatment of localized and locally advanced adrenocortical cancer in children

Introduction. Adrenal cortical carcinoma (ACC) is a rare cancer but is the most common primary cancer in the adrenal gland. Despite the low incidence of ACC the mortality rate ranges from 0.04 to 0.2 %, in the overall structure of cancer mortality. Treatment of ACC is mainly surgical and radical surgical excision is the treatment of choice for local disease stages.Aim of the study — to present our results of surgical treatment of localized and locally advanced ACC in children and to determine the risk factors of relapse.Materials and methods. Twenty-eight patients (median age of 47.8 (06—216) mo.) with localized and locally advanced ACC underwent a retrospectively analysis. Stage I, II, and III revealed in 12 (45 %), 7 (25 %), and 9 (30 %), respectively. In 19 (68 %) cases the secretion of one or more hormone observed. Macroscopically and microscopically complete resection were performed in 26 (93 %) and 23 (82 %) patients, respectively. The median tumor volume was 183 (3.6—1608) cm3 and the median tumor weight was 207.9 (48—710) g.Results. Five-year overall (OS) and relapse-free (RFS) survival were 71 % and 69 %, respectively. OS and RFS according to stage I, II, and III were 100 % vs. 71 % vs. 17 % and 100 % vs. 71 % vs. 14 % respectively. The radical surgical resection and the level of Ki-67 expression influenced significantly the rates of OS and RFS (p < 0.001).Conclusion. The main factor affecting the survival rate of ACC in children with stages I—III is the radical surgical resection. It should be taken into account when planning postoperative therapy. Some of biological characteristics of the tumor could also significantly affect the results of treatment.

A radical tumor resection and vascular plastic with xenopericardium as part of complex therapy in a patient with second relapse of nephroblastoma. Case report and literature review

In spite of a relatively good prognosis of most patients with nephroblastoma, there are some subgroups characterized by different unfavorable prognostic factors, in which the overall prognosis is much worse. In particular, this can be applied to patients with very high risk relapse. As in these cases the tumor is often resistant to most chemotherapy modalities, the quality of surgical control is of utmost importance.We present a case of a 9-year-old patient with second local nephroblastoma relapse involving a large portion of inferior vena cava. During the course of complex therapy a radical surgical resection with vascular plastic by xenopericardium implant was performed. The follow-up, albeit short, yields no signs of disease progression or graft malfunction.This case demonstrates the possibility of successful vascular plastic in a child with relapsed tumor. This method may allow more radical tumor resection.

Interventional Treatment for Cholangiocarcinoma

Cholangiocarcinoma (CCA) is the second most common type of primary liver malignancy. The latest classification includes intrahepatic cholangiocarcinoma and extrahepatic cholangiocarcinoma, with the latter one further categorized into perihilar and distal cholangiocarcinoma. Although surgical resection is the preferred treatment for CCA, less than half of the patients are actually eligible for radical surgical resection. Interventional treatment, such as intra-arterial therapies, ablation, and brachytherapy (iodine-125 seed implantation), has become an acceptable palliative treatment for patients with unresectable CCA. For these patients, interventional treatment is helpful for locoregional control, symptom relief, and improving quality of life. Herein, in a timely and topical manner, we will review these advances and highlight future directions of research in this article.

A retrospective study to evaluate the safety and efficacy of anlotinib plus camrelizumab in management of advanced retroperitoneal sarcoma.

e23545 Background: Several studies have demonstrated the antitumor activity of single-agent anlotinib in the treatment of soft tissue sarcoma (STS). However, the most effective results were from cases of alveolar soft part sarcoma (ASPS), which was a rare subtype in retroperitoneal sarcoma (RPS). The therapy of RPS is still dismal. Thus, we evaluated the effectiveness and safety of anlotinib plus camrelizumab for the treatment of RPS. Methods: We retrospectively reviewed the data of 36 patients with advanced/metastatic RPS in Sarcoma Centre of Peking University Cancer Hospital from July 2019 to February 2021. Anlotinib was given 12mg Qd 2 weeks on and 1 week off, and camrelizumab was transfused 200mg at Q3w. Patients that underwent at least 4 cycles of therapy were enrolled in this study. The primary endpoint is objective response rate (ORR) and evaluated according to the irRECIST 1.1 criteria. Results: The pathologic subtypes of enrolled cases include liposarcoma (LP) (n = 16), leiomyosarcoma (LMP) (n = 5), uncertain differentiation sarcoma (n = 7), fibroblastic and myofibroblastic tumours (n = 4), rhabdomyosarcoma (n = 2) and malignant peripheral nerve sheath tumour (MPNST) (n = 2). Two patients (5.6%) achieved CR and 9 patients (25.0%) achieved PR, with an ORR of 30.6%. Eighteen patients (50.0%) achieved SD and 7 patients (19.4%) were evaluated as PD, with the disease control rate (DCR) of 80.6%. The progression-free rate (PFR) at six months was 60.0%. Five patients underwent further radical surgical resection. The two CR cases were diagnosed as dedifferentiated liposarcoma (DDLP) and MDM2 amplified MPNST respectively, and both patients have been under CR status for over 12 months until now. The only grade 3 or higher treatment related adverse event was hypertension (22.2%). Camrelizumab related reactive cutaneous capillary endothelial proliferation (RCCEP) was not observed in this group of patients. Conclusions: The combination of anlotinib and camrelizumab demonstrated encouraging efficacy and safety in the treatment of RPS. A further clinical study with biomarker exploration should be performed in the future.

Acute-onset diplopia from intracranial hypertension due to torcular herophili obstruction by an hemorrhagic intradiploic epidermoid cyst

Background: Epidermoid cysts are benign slow-growing congenital lesions, constituting approximately 1% of all cranial tumors. Most of these lesions are located intradurally, while about 10–25% of them are located within the diploic spaces. Intradiploic epidermoid cysts are usually discovered incidentally and may remain asymptomatic for many years, but in rare instances, they may grow intracranially and produce brain compression. Sometimes, intradiploic epidermoid cysts may occlude the main cranial venous sinuses causing intracranial hypertension. Case Description: We present the case of a 24-year-old male harboring a paramedian right occipital intradiploic cyst with erosion of both outer and inner bony tables, which occluded the torcular herophili producing a worsening symptomatology with acute-onset diplopia from right sixth cranial nerve palsy; the patient also presented bilateral papilledema, but only reported mild headache and dizziness. Neuroradiological studies evidentiated a lesion compatible with intradiploic epidermoid cyst with intralesional hemorrhagic component, overlying and almost completely occluding the torcular herophili. Considering the fast worsening of symptomatology and the evidence of intracranial hypertension, the patient was operated on immediately after completion of clinical and radiological assessment. The lesion was radically removed with almost immediate reversal of signs and symptoms. Histopathology confirmed the diagnosis of epidermoid cyst with intralesional hemorrhagic components. Conclusion: Intradiploic epidermoid cysts may cause intracranial hypertension by occlusion of main cranial venous sinuses; intralesional hemorrhage may act as precipitating factor in occlusion of the torcular herophili, producing rapidly worsening intracranial hypertension, which requires prompt surgical treatment to reverse symptomatology. Radical surgical resection is necessary to avoid recurrence.