scholarly journals Carcinosarcomas of the larynx: systematic review of the literature of a rare nosologic entity

Author(s):  
Andrea Colizza ◽  
Antonio Gilardi ◽  
Antonio Greco ◽  
Fabrizio Cialente ◽  
Federica Zoccali ◽  
...  

Abstract Purpose Carcinosarcoma, also known as Spindle Cell Carcinoma (SpCC), is a rare type of malignant tumor. Generally, this type of pathology occurs in the urogenital tract, the gastrointestinal tract, respiratory tract and mammary gland; in the larynx, SpCC represents only 2–3% of all malignancies. Due to its rarity, there is currently no generally acceptable treatment guideline for this disease. The aim of this study was to systematically review the literature of SpCC of larynx and report epidemiologic, clinicopathologic and main therapeutic approaches for this entity. Methods A systematic literature review was performed using MEDLINE, EMBASE, PubMed and Scopus databases. For this review, the results were extrapolated in the period between January 1990 to September 2020. Data extraction was performed using a standard registry database. The clinical and pathological staging were recalculated according to the Eight Edition of AJCC Cancer Staging Manual and statistical analyses were performed using SPSS Version 25.0. Results A total of 111 patients affected by laryngeal carcinosarcoma were included. From our review arises that surgery is the main treatment for primary laryngeal carcinosarcoma. In this way, various techniques such as minimally invasive laryngoscopy excision, laser CO2 cordectomy, partial laryngectomy (vertical and horizontal) and total laryngectomy. The role of radiotherapy is still controversial. The overall survival (OS) for T1 stage tumor at 5 years of follow-up is 82.9%, the OS for T2 and T3 tumor is 74% and 73.4%. The OS at 5 years of follow-up is 91.7% for supraglottic tumor, 69.3% for glottic tumor and 50% for transglottic site. Subglottic site is described in only 2 cases [12–13], so the OS at 5 years is not statistically significant. The 5-year overall survival in patients without lymph nodes involvement (N0) is 90.2%, 66.7% and 50%, respectively, for N1 and N2 lesions. Conclusion Primary laryngeal carcinosarcoma is a very rare malignancy. There are no clear guidelines in the management but in the literature, surgery is described as the best modality of therapy; radiation only can be a reasonable alternative with controversial efficacy. The most important prognostic factor is the nodal metastasis.

2021 ◽  
Vol 41 (1) ◽  
pp. 133-136
Author(s):  
Rafael Everton Assunção Ribeiro da Costa ◽  
Danilo Rafael da Silva Fontinele ◽  
Paula Catarina Dalia Rego Medeiros ◽  
Sabas Carlos Vieira

BACKGROUND: Metaplastic breast carcinoma (MBC) is a rare type of breast cancer (0.20–1.00% of all cases). With a more aggressive clinical course, MBC frequently presents as a triple-negative subtype. OBJECTIVE: To describe a case series, analyzing patients survival in four MBC cases. METHODS: The cases were obtained from 532 medical records of breast cancer patients (0.7% of the total). RESULTS: All patients were female. Mean patient age was 49 years (range: 38–60 years). Mean tumor size was 8.9 cm (range: 3.0–15.5 cm). Mastectomy was performed in three cases. One patient had axillary nodal metastasis. All underwent chemotherapy and three received radiation therapy after surgery. CONCLUSIONS: With a mean follow-up of 36 months (range: 10–60 months), one case had a tumor recurrence (25%). Three patients (75%) died from metastatic disease and one (25%) is still alive and free of disease.


2010 ◽  
Vol 49 (03) ◽  
pp. 79-84 ◽  
Author(s):  
J. A. Krämer ◽  
K. W. Schmid ◽  
H. Dralle ◽  
M. Dietlein ◽  
H. Schicha ◽  
...  

SummaryThe Multicentre Study Differentiated Thyroid Cancer (MSDS) collective represents a well defined group of patients with locally aggressive thyroid carcinomas (pT4; AJCC/UICC 1997). The aim of the present study was to compare the survival of patients with minimum and extensive extrathyroidal growth according to the new AJCC/UICC TNM staging system 2009. Patients, methods: The followup data of 347 patients were analysed. Patients were reclassified according to the current AJCC/UICC 2009 classification. The event-free and overall survival was evaluated using Kaplan-Meier analysis. In addition, postoperative complications and status of disease were documented. Results: 327 patients were assigned to stage pT3 and 20 patients to stage pT4a, respectively. Median follow-up was 6.1 years (range 0.04–9.8 years). 92.5% of patients reached complete remission. There were 7.8 % recurrences in the thyroid bed, in locoregional lymph nodes and/or in distant sites. The overall survival was >98% both in pT3 and pT4a patients (p = n. s.). In contrast, the event-free survival was significantly less favourable in pT4a patients (p < 0.001). Using multivariate analysis the following parameters were significant predictors of event-free survival: histological tumour type, degree of extrathyroidal extension and nodal metastasis (p < 0.05). Conclusions: The MSDS patients with locally aggressive differentiated thyroid cancer showed an excellent overall survival during a median follow-up of 6.1 years. According to the current AJCC/UICC 2009 classification, pT3 patients with minimal extra thyroidal extension revealed a significantly better event-free survival than pT4a patients with extensive extrathyroidal growth.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 10073-10073
Author(s):  
O. Merimsky ◽  
V. Soyfer ◽  
A. Goffman ◽  
J. Issakov ◽  
J. Bickels ◽  
...  

10073 Background: Synovial sarcoma (SS) of an extremity or trunk is a relatively rare type of soft tissue sarcoma. SS most commonly affects adolescents and young adults. SS usually originates at an extremity, carries a t(X;18)(p11;q11) translocation, and approached by limb sparing surgery, radiation therapy, and chemotherapy. Methods: A retrospective analysis of clinical and histopathological data of 73 patients with proven SS, treated at the National Unit of Orthopedic Oncology, from January 1991 through December 2004 was performed. Results: At a median follow-up time of 6 years, a local-recurrence was observed in 17.8% of the patients, while systemic recurrence in 35.6% (local-only in 6.8 %, systemic-only in 24.6 %, and combined in 11%). Accumulation of events of local and systemic recurrence following a limb sparing approach, did not reach a plateau even after 192 months from diagnosis. The 10-year local recurrence free survival (LRFS), the 10-year systemic recurrence-free survival (SRFS), and the 10-year overall survival (OS) were 78%, 68%, and 61%, respectively. The median SRFS time was 180 months, while the median LRFS and OS have not been reached yet. LRFS was significantly better for ILP treated patients; SRFS was influenced by a shorter delay in diagnosis. Conclusions: The practical aspects of our observations are the need for long-term follow-up for diagnosis of recurrence, the fact that not all local or distant recurrences are necessarily associated with shortening of overall survival, and the important role of induction ILP with TNF in cases of extremity SS. No significant financial relationships to disclose.


Open Medicine ◽  
2020 ◽  
Vol 15 (1) ◽  
pp. 890-897
Author(s):  
Francesco Stillo ◽  
Federica Ruggiero ◽  
Antonio De Fiores ◽  
Rita Compagna ◽  
Bruno Amato

AbstractBackgroundFirst identified in 2014, fibroadipose vascular anomaly (FAVA) is a very rare type of venous and lymphatic malformation. Marked by tough fibrofatty tissue in the extremities overtaking portions of the muscles, it is associated with constant pain and contracture of the affected extremity. There is a paucity of literature, and no guidelines on treatment procedure are available. This case highlights the role of hybrid treatment with primary ethanol percutaneous ethanol embolization and additional surgery for radicality in excision of FAVA lesions.Case summaryA 9-year-old girl with FAVA underwent the hybrid treatment. The achievements of complete excision, clinical response, and patient satisfaction in long-term follow-up were assessed. Following the hybrid treatment, the patient experienced significant improvement in pain. Concurrent symptoms of physical limitation, leg swelling, and skin hyperesthesia also improved. The clinical benefit, supported by postoperative physiotherapy, was well stabilized at 6-month follow-up, resulting in complete patient satisfaction at 12- and 36-month follow-ups. No major complications were encountered.ConclusionEthanol embolization plus surgery is a safe, effective, and long-term hybrid treatment of symptomatic FAVA lesions.


Viruses ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 904
Author(s):  
Ramin Raul Ossami Saidy ◽  
Irina Sud ◽  
Franziska Eurich ◽  
Mustafa Aydin ◽  
Maximilian Paul Postel ◽  
...  

Patients after LT due to combined HBV/HDV infection are considered to be high-risk patients for recurrence of hepatitis B and D. To date, life-long prophylaxis with hepatitis B immunoglobulin (HBIG) and replication control with nucleos(t)ide analogs (NA) remains standard. We examined the course of 36 patients that underwent liver transplantation from 1989 to 2020 for combined HBV/HDV-associated end-stage liver disease in this retrospective study. Seventeen patients eventually discontinued HBIG therapy for various reasons. Their graft function, histopathological findings from routine liver biopsies and overall survival were compared with those that received an unaltered NA-based standard regimen combined with HBIG. The median follow-up was 204 and 227 months, respectively. The recurrence of HBV was 25% and did not differ between the groups of standard reinfection prophylaxis NA/HBIG (21.1%) and HBIG discontinuation (29.4%); (p = 0.56). No significant differences were found regarding the clinical course or histopathological aspects of liver tissue damage (inflammation, fibrosis, steatosis) between these two groups. Overall, and adjusted survival did not differ between the groups. Discontinuation of HBIG in stable patients after LT for combined HBV/HDV did not lead to impaired overall survival or higher recurrence rate of HBV/HDV infection in this long-term follow-up. Therefore, the recommendation of the duration of HBG administration must be questioned. The earliest time of discontinuation remains unclear.


Author(s):  
Yanhong Wang ◽  
Yi Ouyang ◽  
Jingjing Su ◽  
Lihua Xiao ◽  
Zhigang Bai ◽  
...  

Abstract Objective We used National Cancer Institute’s Surveillance, Epidemiology and End Result database to assess the role of salvage radiotherapy for women with unanticipated cervical cancer after simple hysterectomy. Methods Patients with non-metastatic cervical cancer and meeting inclusion criteria were divided into three groups based on treatment strategy: simple hysterectomy, salvage radiotherapy after hysterectomy and radical surgery. Parallel propensity score-matched datasets were established for salvage radiotherapy group vs. simple hysterectomy group (matching ratio 1: 1), and salvage radiotherapy group vs. radical surgery group (matching ratio 1:2). The primary endpoint was the overall survival advantage of salvage radiotherapy over simple hysterectomy or radical surgery within the propensity score-matched datasets. Results In total, 2682 patients were recruited: 647 in the simple hysterectomy group, 564 in the salvage radiotherapy group and 1471 in the radical surgery group. Age, race, histology, grade, FIGO stage, insured and marital status and chemotherapy were comprised in propensity score-matched. Matching resulted in two comparison groups with neglectable differences in most variables, except for black race, FIGO stage III and chemotherapy in first matching. In the matched analysis for salvage radiotherapy vs. simple hysterectomy, the median follow-up time was 39 versus 32 months. In the matched analysis for salvage radiotherapy vs. radical surgery, the median follow-up time was 39 and 41 months, respectively. Salvage radiotherapy (HR 0.53, P = 0.046) significantly improved overall survival compared with simple hysterectomy, while salvage radiotherapy cannot achieve similar overall survival to radical surgery (HR 1.317, P = 0.045). Conclusions This is the largest study of the effect of salvage radiotherapy on overall survival in patients with unanticipated cervical cancer. Salvage radiotherapy can improve overall survival compared with hysterectomy alone, while cannot achieve comparable survival to radical surgery.


Author(s):  
Osman Öcal ◽  
Kerstin Schütte ◽  
Juozas Kupčinskas ◽  
Egidijus Morkunas ◽  
Gabija Jurkeviciute ◽  
...  

Abstract Purpose To explore the potential correlation between baseline interleukin (IL) values and overall survival or objective response in patients with hepatocellular carcinoma (HCC) receiving sorafenib. Methods A subset of patients with HCC undergoing sorafenib monotherapy within a prospective multicenter phase II trial (SORAMIC, sorafenib treatment alone vs. combined with Y90 radioembolization) underwent baseline IL-6 and IL-8 assessment before treatment initiation. In this exploratory post hoc analysis, the best cut-off points for baseline IL-6 and IL-8 values predicting overall survival (OS) were evaluated, as well as correlation with the objective response. Results Forty-seven patients (43 male) with a median OS of 13.8 months were analyzed. Cut-off values of 8.58 and 57.9 pg/mL most effectively predicted overall survival for IL-6 and IL-8, respectively. Patients with high IL-6 (HR, 4.1 [1.9–8.9], p < 0.001) and IL-8 (HR, 2.4 [1.2–4.7], p = 0.009) had significantly shorter overall survival than patients with low IL values. Multivariate analysis confirmed IL-6 (HR, 2.99 [1.22–7.3], p = 0.017) and IL-8 (HR, 2.19 [1.02–4.7], p = 0.044) as independent predictors of OS. Baseline IL-6 and IL-8 with respective cut-off values predicted objective response rates according to mRECIST in a subset of 42 patients with follow-up imaging available (IL-6, 46.6% vs. 19.2%, p = 0.007; IL-8, 50.0% vs. 17.4%, p = 0.011). Conclusion IL-6 and IL-8 baseline values predicted outcomes of sorafenib-treated patients in this well-characterized prospective cohort of the SORAMIC trial. We suggest that the respective cut-off values might serve for validation in larger cohorts, potentially offering guidance for improved patient selection.


2021 ◽  
Vol 32 ◽  
pp. S125-S126
Author(s):  
G. Calderillo-Ruiz ◽  
C. Diaz ◽  
H. Lopez Basave ◽  
E. Ruiz-Garcia ◽  
A. Apodaca ◽  
...  

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii390-iii391
Author(s):  
Aaron Mochizuki ◽  
Anna Janss ◽  
Sonia Partap ◽  
Paul Fisher ◽  
Yimei Li ◽  
...  

Abstract INTRODUCTION Medulloblastoma is one of the most common malignant brain tumors in children. To date, the treatment of average-risk (non-metastatic, completely resected) medulloblastoma includes craniospinal radiation therapy and adjuvant chemotherapy. Modern treatment modalities and now risk stratification of subgroups have extended the survival of these patients, exposing the long-term morbidities associated with radiation therapy. METHODS We performed a single-arm, multi-institution study, seeking to reduce the late effects of treatment in patients with average-risk medulloblastoma prior to advances in molecular subgrouping. To do so, we reduced the dose of craniospinal irradiation by 25% to 18 gray with the goal of maintaining the therapeutic efficacy as described in CCG 9892 with maintenance chemotherapy. RESULTS 28 patients aged 3–30 years were enrolled across three institutions between April 2001 and December 2010. Median age at enrollment was 9 years with a median follow-up time of 11.7 years. The 3-year relapse-free (RFS) and overall survival (OS) were 78.6% (95% CI 58.4% to 89.8%) and 92.9% (95% CI 74.4% to 98.2%), respectively. The 5-year RFS and OS were 71.4% (95% CI 50.1% to 84.6%) and 85.7% (95% CI 66.3% to 94.4%), respectively. Toxicities were similar to those seen in other studies; there were no grade 5 toxicities. CONCLUSIONS Given the known neurocognitive adverse effects associated with cranial radiation therapy, studies to evaluate the feasibility of dose reduction are needed. In this study, we demonstrate that select patients with average-risk medulloblastoma may benefit from reduced craniospinal radiation dose of 18 gray without impacting relapse-free or overall survival.


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