Rare Tumors最新文献

{"title":"Extraskeletal myxoid chondrosarcoma: A case series and review of the literature","authors":"Michael P. Fice, Linus Lee, Pavan Kottamasu, Abdullah Almajnooni, Matthew R. Cohn, Charles A. Gusho, S. Gitelis, Alan T. Blank","doi":"10.1177/20363613221079754","DOIUrl":"https://doi.org/10.1177/20363613221079754","url":null,"abstract":"Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue sarcoma (STS) that accounts for less than 3% of all soft tissue tumors. The conventional treatment for primary EMC is wide local excision with or without radiation therapy. Materials and Methods This study was a retrospective review of all EMC cases treated within a single institution between 1992 and 2019. EMC was diagnosed using a combination of histologic morphology and immunostaining, with confirmatory fluorescent in situ hybridization. Overall survival (OS) and disease-specific survival (DSS) were defined using Kaplan–Meier analysis. Results Fifteen patients were evaluated, including 11 males and four females. The average age at presentation was 51.7 ± 20.4 years and the mean follow-up time was 61.5 months (range, 5–286 months). The average resected tumor size at largest dimension was 7.14 cm (range, 2.4–18.7). Twelve of fifteen (80%) patients underwent wide local excision, and nine of the twelve (75%) underwent local radiation therapy. The 1-, 5-, and 10-year OS was 80% (95% CI, 59.8–100), 72% (95% CI, 48.5–95.5), and 72% (95% CI, 48.5–95.5), respectively. The 1-, 5-, and 10-year DSS was 92.3% (95% CI, 77.8–100), 83.1% (95% CI, 61.5–100), and 83.1% (95% CI, 61.5–100), respectively. At last follow-up, 11 patients were alive and ten (90.9%) were disease free. Conclusions Extraskeletal myxoid chondrosarcoma is a very rare STS most often seen in males and in the extremities. Our cohort was too small to provide meaningful statistical analysis; however, we observed lower rates of local recurrence in patients treated with radiation.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43245665","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pelvic retroperitoneal pleomorphic hyalinizing angiectatic tumor: A case report and review of literature.","authors":"Mehdi Salehipour,&nbsp;Mohammad Hossein Anbardar,&nbsp;Bita Geramizadeh,&nbsp;Hamed Jafari,&nbsp;Ali Zare,&nbsp;Ali Adib","doi":"10.1177/20363613221103751","DOIUrl":"https://doi.org/10.1177/20363613221103751","url":null,"abstract":"<p><p>Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of the soft tissue, usually located in lower extremities. There are rarely tumors reported in other anatomic locations. Herein, we report retroperitoneal PHAT in a male patient. A 41-year-old man was referred to our clinic due to an incidentally found retroperitoneal mass. Computed tomography (CT) scan showed a solid hypoechoic lesion containing fat component and calcified elements measuring about 80*72*45 mm in the right lower quadrant (RLQ) of the abdomen. Magnetic resonance imaging (MRI) showed circumscribe lesion measuring about 60 x 48 mm with partial enhancement and fat component. In pelvic exploration, a large mass was found that had encased the right external iliac artery and vein. Therefore, the mass and its surrounding iliac vessels were excised and removed en block. Then, the external iliac vessels were reconstructed with Gortex graft. No recurrence was found in 1 month and 3 months post-operation follow up. We report a pelvic retroperitoneal PHAT as a rare location of this tumor. It seems that PHAT must be considered in differential diagnosis in patients with soft tissue tumors in the pelvic cavity.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/51/60/10.1177_20363613221103751.PMC9150221.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10252651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary leiomyosarcoma of the colon with synchronous liver metastasis","authors":"D. Massaras, E. Kontis, K. Stamatis, E. Zampeli, D. Myoteri, Elias Primetis, E. Pantiora, G. Fragulidis","doi":"10.1177/20363613221080549","DOIUrl":"https://doi.org/10.1177/20363613221080549","url":null,"abstract":"Leiomyosarcomas (LMS) are mesenchymal tumors of smooth muscle origin. Approximately 20% of leiomyosarcomas are found in the GI tract, and account for 1–2% of GI malignancies. Within the gastrointestinal tract, the small intestine is the most common site of presentation followed by the colon. They are often diagnosed incidentally during abdominal pain investigation, and they usually present in the fifth decade of life. In the past, the diagnostic differentiation between leiomyosarcomas of gastrointestinal tract and gastrointestinal stromal tumors (GISTs) was very challenging as they share common microscopic appearance. Nowadays, these tumors are diagnosed by immunohistochemical methods as they are positive for smooth muscle markers actin (SMA) and desmin, negative for GIST markers such as receptor tyrosine kinase (KIT), CD34, DOG1, and negative for the schwannoma marker S100 protein. In the current literature, most cases of intestinal leiomyosarcomas are localized in the small intestine, and there are no reports of synchronous liver metastases at the time of presentation of the primary tumor. Herein, we present a patient who was admitted in our department, with the diagnosis of primary leiomyosarcoma of the colon and synchronous liver metastasis.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48957682","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Giant teratoma of the pancreas expanding to the mediastinum: Rare tumor and literature review.","authors":"Nikolaos Tasis,&nbsp;Antonia A Prountzopoulou,&nbsp;Evangelia Skafida,&nbsp;Maria Arnaouti,&nbsp;Theodoros Tsirlis,&nbsp;Aris Plastiras,&nbsp;Dimitrios K Manatakis,&nbsp;Niki Arnogiannaki,&nbsp;Dimitrios P Korkolis","doi":"10.1177/20363613221147470","DOIUrl":"https://doi.org/10.1177/20363613221147470","url":null,"abstract":"<p><p>Pancreatic mature cystic teratomas are very rare with limited cases found in the literature. These lesions raise a diagnostic challenge and complicate the surgical approach not only because of their anatomic position but also because of their ever-growing size. An elusive diagnosis, usually leads to the operative theatre where surgical resection takes place. We present a rare case of a large pancreatic cystic teratoma extending into the mediastinum in a 29-year-old woman which was succesfully managed with en-bloc distal pancreatectomy and spleenectomy.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3b/bb/10.1177_20363613221147470.PMC9806373.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10494337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A rare case report of a myxoid liposarcoma arising from the broad ligament.","authors":"Farah Sassi,&nbsp;Ghada Sahraoui,&nbsp;Lamia Charfi,&nbsp;Zemni Ines,&nbsp;Karima Mrad,&nbsp;Raoudha Doghri","doi":"10.1177/20363613221148839","DOIUrl":"https://doi.org/10.1177/20363613221148839","url":null,"abstract":"<p><p>Myxoid liposarcoma (MLPS) is the second most prevalent subtype of liposarcoma. It is usually found in the deep tissues of the lower limbs and rarely in gynecologic tract. Herein we present the second case in the English literature of a primary MLPS arising from the broad ligament which was thought to be a borderline ovarian tumor. The aim is to discuss its clinical and pathological characteristics. A 42-year-old woman presented with pelvic pain for the last 6 months. Magnetic resonance imaging was not specific. She underwent a surgical resection of the tumor mass, and pathological examination confirmed the diagnosis of MLPS deriving from the broad ligament. She received radiotherapy and the patient is doing well at 3 months follow-up. The clinical aspects, pathological diagnosis, prognosis, and therapy approach of broad ligament MLPS are all poorly understood. Complete surgical resection with or without radiotherapy is the mainstay of treatment in located MLPS.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4b/05/10.1177_20363613221148839.PMC9793064.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10446283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"EXPRESSION OF CONCERN: The first case report of primary thyroid teratocarcinosarcoma: An analog to sinonasal teratocarcinosarcoma","authors":"","doi":"10.1177/20363613221101702","DOIUrl":"https://doi.org/10.1177/20363613221101702","url":null,"abstract":"[This corrects the article DOI: 10.1177/20363613211043662.].","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42188294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and paraclinical features, outcome, and prognosis of ovarian granulosa cell tumor: A retrospective study of 28 Vietnamese women.","authors":"Duc Thanh Le,&nbsp;Tu Anh Do,&nbsp;Linh Ly Thi Nguyen,&nbsp;Kien Hung Do,&nbsp;Chu Van Nguyen","doi":"10.1177/20363613221148547","DOIUrl":"https://doi.org/10.1177/20363613221148547","url":null,"abstract":"<p><p><b>Background:</b> Granulosa cell tumor of the ovary is a rare disease and presents with two clinically and molecularly distinct subtypes: the juvenile and the adult type. GCT is considered as a malignant tumor with an indolent course and a tendency toward late recurrence. <b>Purpose</b>: To assess the clinical and paraclinical features, treatment findings, survival outcomes, and explored the prognostic factors in the granulosa cell tumor. <b>Methods</b>: The current study was conducted on 28 GCT patients who had surgical operations and adjuvant chemotherapy (stage IC-IV) by applying a retrospective cohort analysis. The clinical and paraclinical characteristics were recorded. Recurrent status was evaluated for analysis with clinical and paraclinical features and survival. All GCT patients' survival were analyzed by using Kaplan-Meier and Log-Rank models. <b>Results</b>: 17.9% of patients experienced a relapse and two patients died due to disease. The mean time from initial diagnose to recurrence was 40.21 months. The 5-year OS and DFS of stage I-II were 100% and 80.8%, and of stage III were 50% and 25%, respectively. In survival analyses, using the log-rank test, age ≥50 years, irregular menstruation, stage I-II, and absence of residual lesion were all significant predictors for the improved DFS. Stage I-II and absence of residual lesion were associated significantly with better OS. Mean of age, FIGO stage, and residual lesion during surgery had significant differences to recurrent rate (p < <0.05). The multivariate model revealed that these factors didn't remain as an independent prognostic variable. <b>Conclusion</b>: FIGO stage and residual lesion during surgery had significant differences in survival and recurrent rate.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/56/12/10.1177_20363613221148547.PMC9793063.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10455193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Title: Clinico-pathological and survival profiles of orbito-ocular tumors in Enugu Nigeria. Is there an emerging trend?","authors":"N. Uche, O. Okoye, O. Okoye, I. Oguegbu, E. Uche","doi":"10.1177/20363613221079763","DOIUrl":"https://doi.org/10.1177/20363613221079763","url":null,"abstract":"Background Orbito-ocular tumors are rare, but they cause significant morbidity and mortality. There are reported variations in their pattern of presentation and frequency of occurrence. Objective To evaluate the occurrence, clinico-pathologic spectrum, and patterns of orbito-ocular neoplasms as well as the treatment and outcome profiles. Methods A 5-year retrospective analysis of consecutive patients treated for orbito-ocular tumors. We evaluated patterns of occurrence, clinic-pathological concordance, and outcome of treatment. Associations were evaluated with chi square and confidence interval. Data analysis was performed using SPSS for windows version 23 and inferences were judged using the 95% level of significance. Results Among 100 patients with orbito-ocular masses, 62 were histologically confirmed. The female to male ratio (F:M) was 1.0. Their ages ranged from 7 months to 93 years, mean = 33.4 ± 2.7 years. There was an age variation among tumor types. Patients with squamous cell carcinoma (SCC) had a mean age of 46.4 years, while for retinoblastoma the mean age was 3.09 years. All patients with retinoblastoma had proven macroscopic orbital extension. SCC was the most common tumor type (n = 19), however, among children, retinoblastoma (n = 11) was more common. Melanocytic nevus, sebaceous gland carcinoma, and adenocarcinoma of lacrimal gland (n = 6, n = 5, n = 5), respectively, among other tumors were treated. Concordance between clinical and histopathological diagnoses was obtained among 30 (48.3%) cases. 1-year and 3-year survival for retinoblastoma was 90% and 72.9%, respectively, and 78.9% and 68.4% for SCC. Conclusion SCC is currently the most common orbito-ocular tumor in our setting.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43326354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quality of life of patients with soft tissue sarcoma treated with doxorubicin in the ANNOUNCE phase III clinical trial","authors":"B. V. Van Tine, A. Krarup-Hansen, L. Hess, A. A. Abdul Razak, V. Soldatenkova, Jennifer Wright, S. Park","doi":"10.1177/20363613221100033","DOIUrl":"https://doi.org/10.1177/20363613221100033","url":null,"abstract":"Background: Patient-reported outcomes (PROs), including health-related quality of life, are recommended to be routinely collected in clinical trials, but data are limited from trials of sarcoma patients. In this analysis, pooled PRO data are reported from patients with advanced or metastatic soft tissue sarcoma (STS) enrolled to the ANNOUNCE phase III trial of doxorubicin-based therapy. Methods: ANNOUNCE was a phase III trial that randomized 509 patients with STS to receive up to eight cycles of doxorubicin with olaratumab or placebo, followed by single-agent olaratumab or placebo. Dexrazoxane was allowed at any cycle of treatment. Participants completed the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ-C30, which is scored 0–100), and Brief Pain Inventory Short Form Modified (mBPI-sf, scored from 0–10) at each treatment cycle. A descriptive analysis of the longitudinal data was conducted overall and by cumulative dose of doxorubicin received to inform the clinical care of patients with STS. Worsening on the QLQ-C30 was defined as a change of 10 points or more at any post-baseline assessment. Worsening on the mPBI-sf was defined as an increase of ≥2 points from baseline. Results: The majority of participants completed the baseline and at least one subsequent PRO assessment within the trial (n = 460, 90.4% EORTC QLQ-C30; n = 454, 89.2%, mBPI-sf). Patients with STS enrolled to the ANNOUNCE trial had clinically meaningful problems with physical function and pain before initiating doxorubicin. Overall, those with fewer symptoms or better function at baseline received higher cumulative doxorubicin dose throughout the study. At baseline, mean QLQ-C30 fatigue was 29.9 with a median time to first worsening of 0.9 months, and mean nausea/vomiting was 6.5 with 1.4 months until worsening; mean physical function was 78.3 with median time to worsening of 2.1 months and mean health status was 66.8 with median time to first worsening of 1.6 months. Median time to worsening of pain was 7.9 months. Conclusion: Patients with advanced or metastatic sarcoma reported a relatively rapid decline in PROs during doxorubicin-based treatment, with patients with poorer symptoms at baseline (specifically fatigue), subsequently receiving less doxorubicin therapy. The availability of detailed summary data from the patient perspective during doxorubicin-based treatment may inform future care of these patients and can provide a resource for the development of PRO endpoints in future trials.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47755898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Polymorphous low-grade neuroepithelial tumor of the young: Rare tumor and review of the literature","authors":"Ali H. Palejwala, Christen M. O’Neal, Michael R Quinton, J. Battiste, J. Peterson, I. Dunn","doi":"10.1177/20363613221083360","DOIUrl":"https://doi.org/10.1177/20363613221083360","url":null,"abstract":"Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described low-grade neuroepithelial tumor with an infiltrative growth pattern and oligodendrocyte-like cells that are CD34 immunopositive. Correlating histology and results from molecular testing is critical to correctly diagnosing PLNTY, as its histologic appearance is similar to oligodendrogliomas and shares genetic abnormalities common to other low-grade epilepsy associated tumors (LEATs). In this case report, we describe a 31-year-old female with intractable epilepsy found to have a temporal mass and diagnosed with PLNTY after histopathologic and molecular testing. We describe the radiographic, histologic, and genetic features in relation to the epileptic and oncologic outcomes for this patient. Then, we compare these features and outcomes to other cases of PLNTY described in the literature.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43570520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}