Materiały źródłowe

• #1 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The 2020 World Health Organization classification of STSs incorporates UPS under malignant tumors of uncertain differentiation. According to the Surveillance, Epidemiology and End Results (SEER) program, UPS accounted for 17.1% out of 26,758 cases and was the second most common STS after leiomyosarcoma, regardless of the primary tumor site. Males showed considerably increased incidence rates than females, and white males were more commonly affected than black males. Incidence also linearly increased with advanced age, which was higher beyond the 6th decade of life. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Follow-up visits are important to detect local recurrences or metastasis. Physical examination of stage I tumors should be performed at 3-to-6-month intervals for the first two years and then annually. Stage II to IV disease must be followed every 2-to-6 months for 2 to 3 years, then every 6 months for 2 years, and annually thereafter.

• #2 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcomas (UPS) are included in the World Health Organization’s 2020 categorization of soft-tissue sarcomas (STS) as malignant tumors of unclear distinction. Undifferentiated pleomorphic sarcomas (UPS) accounted for 17.1 % of the 26,758 cases and is the second most prevalent soft-tissue sarcomas (STS) after leiomyosarcoma, independent of the underlying tumor location. Males had much higher incidence rates than females, and white males were impacted more frequently than black males. Incidence also is the highest during the sixth decade of life.

• #3 Incidence of Undifferentiated Pleomorphic Sarcoma (UPS) in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11537747/

  UPS has been suggested to be among the most common types of STS, but the real incidence of UPS in US is not clear. […] The current project aims to address and narrow down the existing gap in incidence of UPS in the US. Our study could not provide the precise estimate on UPS incidence in US due to the lack of designated codes of UPS; however, by implementing multiple ways of estimates, this study can provide our best estimate on the likely range of UPS incidence and explore the impact based on different definitions. The project endeavors will provide tangible solutions that will significantly reduce the gap and pave the way for enhanced UPS epidemiology. […] During 2016-2020, the crude incidence of UPS was 0.06 per 100,000 persons when using MFH only to define UPS, representing 1.8% of all STS. The crude incidence and proportion were 0.38 per 100,000, and 11.2% when including fibrosarcoma NOS, fibromyxosarcoma, fascial fibrosarcoma, and myxosarcoma in the second definition and were 0.67 per 100,000 and 20.0% when further including sarcoma NOS.

• #4

  https://typeset.io/papers/incidence-of-undifferentiated-pleomorphic-sarcoma-ups-in-the-3k12989829oh

  This study estimates the incidence of undifferentiated pleomorphic sarcoma (UPS) in the US using 2000-2020 SEER data, finding varying rates (0.06-0.67 per 100,000) and a decline in incidence over two decades, highlighting the need for standardized diagnosis and classification guidelines. […] The incidence varied widely across the three definitions with 0.06 per 100,000 persons for the least inclusive definition and 0.67 per 100,000 persons for the most inclusive definition in 2016-2020, making it challenging to estimate the exact incidence of UPS. […] Regardless, all the incidences decreased between 2000 and 2020. Guidelines in UPS diagnosis and classification need to be better implemented in the US.

• #5 Orphanet: Undifferentiated pleomorphic sarcoma

  https://www.orpha.net/en/disease/detail/2023

  An aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis. […] UPS ranks the 4th most common soft tissue sarcoma with a slight male preponderance. The incidence has been evaluated to be close to 0.8-1 new case per 100000 per year in one European series. […] A 5-year overall survival rate of 48% has been reported for patients with head and neck tumors versus 77% for patients with tumors arising on the trunk and extremities. The childhood variant appears have better prognosis.

• #6 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a rare type of soft tissue cancer that spreads quickly. Overall, UPS is rare, affecting fewer than 5,000 people in the U.S. But its one of the three most common soft tissue sarcomas, along with liposarcoma and leiomyosarcoma. […] Undifferentiated pleomorphic sarcoma treatment depends on the size of the tumor and whether the cancer has spread. The main treatment for early-stage UPS is typically surgery to remove the growth, most often combined with radiation therapy. […] Life expectancy varies depending on the size and stage of the sarcoma. The five-year survival rate for high-grade UPS is about 60%. That means that around 60% of people diagnosed with this condition are still alive five years later. The 10-year survival rate is 48%. […] Its possible to cure UPS, especially during the early stages. There still may be treatment options in later stages, which can prolong your quality of life. Like any type of cancer, early detection and treatment increase your chances for successful outcomes.

• #7 Incidence of Undifferentiated Pleomorphic Sarcoma (UPS) in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11537747/

  There are little data on the incidence of UPS, and historical literature on MFH may have significant limitation due to evolving STS classification guideline. […] The current incidence of UPS in the US is likely higher than 0.06 per 100,000 but probably not higher than 0.67 per 100,000 persons. […] In summary, the incidence of UPS continued to decrease in the past 2 decades in the US. However, it is challenging to estimate the real incidence of UPS due to potential confusion in its definition and classification. Guidelines in UPS diagnosis and classification need to be better implemented throughout clinical practices in the US.

• #8 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  Despite its low incidence—increasing from 3 cases per 100,000 people in 2013 to three 3 per 45,000 currently—UPS demonstrates a worrying trend towards a gradual increase in occurrence. […] The primary sites for metastatic spread are typically the lungs, as demonstrated in our case report, and less frequently, the liver. […] Approximately 50% of all UPS cases are found in the lower extremities, and another 20% in the upper extremities, predominantly affecting middle-aged to elderly males. […] Conversely, the occurrence of UPS in the retroperitoneum is notably rare, with our case representing only the eighth documented instance. […] Diagnosing undifferentiated pleomorphic sarcoma poses significant challenges due to its rarity and the nonspecific nature of its imaging characteristics.

• #9 Undifferentiated pleomorphic sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/undifferentiated-pleomorphic-sarcoma-1?lang=us

  Typically, undifferentiated pleomorphic sarcomas occur in adults (range 32-80; mean 59 years) with a slight male predilection (M:F 1.2:1). […] They are the most frequent soft tissue sarcoma to occur as a result of radiotherapy and are also seen on a background of Paget disease. Secondary transformation into malignant sarcomas (including undifferentiated pleomorphic sarcoma) has also been reported in fibrous dysplasia, giant cell tumor, enchondroma, chronic osteomyelitis, and osteonecrosis. […] Most undifferentiated pleomorphic sarcomas are high grade (3 and 4) and are aggressive in their biological behavior. They frequently metastasize (30-50% at diagnosis) and locally recur despite aggressive treatment. The overall 5-year survival is between 25-70%. […] Prognostic factors include tumor size: smaller being better, location: superficial is better, distal is better, histological grade, histological subtype: the myxoid subtype has a better prognosis compared to the storiform-pleomorphic subtype.

• #10 Surgical & Cosmetic Dermatology | Undifferentiated pleomorphic sarcoma: a case report

  http://www.surgicalcosmetic.org.br/details/822/en-US/undifferentiated-pleomorphic-sarcoma–a-case-report

  Undifferentiated pleomorphic sarcoma (UPS) is the fourth most important soft tissue sarcoma. […] Regarding its epidemiology, it affects mostly men (two thirds) in relation to women, in the age group of 50 to 70 years (most affected age group). […] It is more frequent in the lower limbs, but 10% to 15% occur in the head and neck. […] Regarding its evolutionary trend, it presents a local recurrence of 19% to 31%, and a metastasis index of 31% to 35% (with a frequency of 90% in the lungs, 85% in the bones, and 1% in the liver). Its five-year survival rate is 65% to 70%.

• #11 Undifferentiated Pleomorphic Sarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/undifferentiated-pleomorphic-sarcoma/

  Undifferentiated pleomorphic sarcoma (UPS) is a rare, high-grade bone tumor characterized by pleomorphic cells and a lack of specific differentiation, often diagnosed by exclusion. […] UPS of bone is rare, representing < 2% of all primary malignant bone tumors. Males are more frequently affected than females. There is a broad age distribution, from the second to eighth decades of life, but most patients are aged > 40 years. Only 10–15% of cases occur in patients aged < 20 years. [...] Patients with localized disease and adequate therapy have a 5-year survival rate of 50–67%. Pulmonary metastases are common, occurring in approximately 35–50% of cases. Secondary UPS and metastatic disease are associated with a poorer prognosis. Incomplete expression of myogenic markers is not thought to affect the prognosis.

• #12 Frontiers | Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2017.00091/full

  Soft-tissue sarcomas account for 0.7% of all malignant tumors, with an incidence rate of 3 per 100,000 persons/year. […] UPS comprises a series of high-grade soft-tissue sarcomas often associated with poor prognosis. […] UPS appears typically in patients that are approximately 50–70 years of age, with a slight male predominance and can arise in any part of the body, but most commonly in the extremities. […] Local recurrence of the tumor in the same location occurs in 25% of all the patients with soft-tissue sarcomas but it is lower in soft-tissue extremity sarcomas. […] Most recurrences develop in the first 2 years after treatment but can occur at any time during the life of the patient. […] Deep tumors recur in about 40%, and 50% of them metastasize. […] The most frequent metastatic presentation is pulmonary disease (90%) being extra-pulmonary sites uncommonly involved lymph nodes (10%), bone (8%), and liver (1%).

• #13 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/119459/html

  There are more reported cases of undifferentiated pleomorphic sarcoma (UPS) in the chest for women than men. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Current literature suggests that radiation-associated UPS occurs in 5.2% of UPS cases, but these case reports demonstrate an occurrence of 5/22 for secondary malignancies (22.7%). […] This study illustrates that are more case reports of women with UPS of the chest wall despite men tending to develop UPS more often in general. […] Additionally, RT and chemotherapy are often recommended in the treatment UPS, but this study found no difference in the recurrence or metastasis of UPS in the chest wall between patients receiving therapy and those who did not.

• #14 A Trp53fl/flPtenfl/fl mouse model of undifferentiated pleomorphic sarcoma mediated by adeno-Cre injection and in vivo bioluminescence imaging | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0183469

  One of the most common genetic alterations in sarcomas with complex karyotypes is tumor protein 53 (TP53) inactivation, either via somatic mutation, deletion, or alterations in TP53 pathway genes such as CDKN2A and MDM2. […] The experimental mice were created by crossing ROSA26 lox-stop-lox luciferase, Ptenfl/fl and Trp53fl/fl mice together, to create offspring with three homozygous floxed alleles. […] Both SQ and IM injection of Ad CMV-Cre virus into Trp53fl/flPtenfl/fl mice resulted in primary soft tissue sarcomas arising at the site of injection with 100% penetrance. […] Examination of histology from both SQ- as well as IM-arising tumors revealed findings consistent with UPS, demonstrating atypical spindle cells with no identifiable line of differentiation. […] Fourteen primary tumors were analyzed histologically, and while 13 of these (93%) were UPS, one tumor (7%) was pleomorphic rhabdomyosarcoma (PRMS).

• #14 A Trp53fl/flPtenfl/fl mouse model of undifferentiated pleomorphic sarcoma mediated by adeno-Cre injection and in vivo bioluminescence imaging | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0183469

  The presence of small mature lymphocytes suggested the presence of immune surveillance in the immunocompetent mouse model of UPS. […] To test the hypothesis that sarcomas in this mouse model demonstrated immune tolerance, we performed immunohistochemistry for PD-L1 (B7-H1) and demonstrated 71% of the tumors to upregulate PD-L1. […] These findings suggest that this mouse model can be used as a pre-clinical model to test the efficacy of anti PD-1/PD-L1 therapy (e.g. pembrolizumab) in UPS.

• #15 Post-operative surveillance in soft tissue sarcoma: using tumorspecific recurrence patterns to direct approach – Zaidi – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/20903/html

  Significant challenges exist in creating surveillance recommendations for soft tissue sarcomas (STS) given the vast heterogeneity of recurrence patterns between histologic subtypes. […] Although surveillance protocols have been developed in parallel with our growing knowledge of cancer biology, many of these surveillance programs lack evidence for their effectiveness in detecting local or distant recurrences at a treatable stage. This is especially true in soft tissue sarcoma (STS), where the complex heterogeneity of the disease presents particular challenges in crafting effective surveillance strategies. […] The natural history of STS is determined largely by the histologic subtype of tumor and the anatomic site of origin. […] For patients with resected stage 1A/1B extremity/superficial trunk STS, postoperative surveillance includes a history and physical exam every 3 to 6 months for 2 to 3 years and then annually afterwards with a consideration for chest imaging, as well as baseline postoperative and periodic imaging of the primary site of disease based on the estimated risk of locoregional recurrence.

• #15 Post-operative surveillance in soft tissue sarcoma: using tumorspecific recurrence patterns to direct approach – Zaidi – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/20903/html

  Unique obstacles exist in creating surveillance strategies for STS due mainly to the diverse natural history and recurrence patterns between tumor subtypes. In recent years however, multiple groups have compiled extended follow-up data on the specific histologic subtypes of STS. In light of this data, we propose using tumor-specific protocols for postoperative surveillance centered around the histologic subtype of each tumor.

• #16 Undifferentiated Pleomorphic Sarcoma | Orthopedic Surgery | Patient Care | Montefiore Einstein

  https://montefioreeinstein.org/patient-care/services/orthopedics/specialties/orthopedic-oncology/pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that arises in soft tissue. UPS represents about 10 percent of all adult soft-tissue sarcomas, making it one of the most common subtypes. […] These tumors have a substantial likelihood of metastasizing. They typically develop in older adult patients and are relatively rare in the pediatric population. […] Following the completion of treatment, patients typically require close monitoring for a period of five years. This includes physical examinations and periodic imaging studies. Many patients continue to follow up even after five years, though at a reduced rate.

• #17 Analysis of prognostic factors of undifferentiated pleomorphic sarcoma and construction and validation of a prediction nomogram based on SEER database | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00810-z

  Undifferentiated pleomorphic sarcoma (UPS) is considered one of the most common types of soft tissue sarcoma (STS). […] The purpose of this study is to construct and validate a nomogram for predicting OS in UPS patients at 3, 5 years after the diagnosis. […] According to the inclusion and exclusion criteria, 1079 patients with UPS were screened from the SEER database and randomly divided into the training cohort (n=755) and the validation cohort (n=324). […] The ending events in this study were death due to various causes. The time from diagnosis of UPS to death due to various causes is called overall survival. The ending indicators were 3-year and 5-year overall survival rates of patients with UPS. […] In this study, the overall survival of patients with UPS was 83 months, and the 3- and 5-year OS were 65.5% and 55.6%, respectively.

• #17 Analysis of prognostic factors of undifferentiated pleomorphic sarcoma and construction and validation of a prediction nomogram based on SEER database | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00810-z

  The results of this study show that for patients with UPS, surgery reduced the risk of cancer-specific death by 59% and the overall risk of death by 61%, and radiotherapy reduced the risk of cancer-specific death by 24% and the overall risk of death by 29%, demonstrating that surgery and radiotherapy can improve the prognosis of UPS patients to some extent. […] By applying the KM method and Cox regression analysis, this study determined nine independent prognostic factors: age, tumor site, tumor size, tumor depth, N stage, M stage, tumor grade, surgery, and radiotherapy. […] The nomogram can individually predict 3-year and 5-year OS of UPS patients, with good discrimination, accuracy and clinical utility. It can provide a reference for clinicians and patients to make better clinical decisions.

• #18 Undifferentiated Pleomorphic Sarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/undifferentiated-pleomorphic-sarcoma/

  Undifferentiated pleomorphic sarcoma is a rare cancer that usually forms in soft tissue in the legs, arms or abdomen, but it can form in any soft tissue or bone anywhere in the body. […] Also called malignant fibrous histiocytoma or undifferentiated sarcoma, this aggressive cancer can grow fast and often spreads to other parts of the body, including the lungs. […] The survival rate for undifferentiated pleomorphic sarcoma is 60% at five years and 48% at 10 years, according to a 2019 study. As with all cancers, early detection improves the survival rate. By their nature, long-term survival rates don’t reflect the latest advancements in treatment and may underestimate current survival chances. […] Undifferentiated pleomorphic sarcoma occurs at any age over 2 years and has no known cause or risk factors.

• #19 Incidence of Undifferentiated Pleomorphic Sarcoma (UPS) in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11537747/

  The classification of undifferentiated pleomorphic sarcoma (UPS) has been evolving with advances in immunohistochemistry and genomic profiling over the past 20 years. There is a lack of current information on UPS incidence. Due to the lack of designated histology codes for UPS in the Surveillance, Epidemiology, and End Results (SEERs) program, we estimated UPS incidence by three different definitions based on clinical opinions using the 2000-2020 data from 22 registries of the SEER program. The incidence varied widely across the three definitions with 0.06 per 100,000 persons for the least inclusive definition and 0.67 per 100,000 persons for the most inclusive definition in 2016-2020, making it challenging to estimate the exact incidence of UPS. Regardless, all the incidences decreased between 2000 and 2020. Guidelines in UPS diagnosis and classification need to be better implemented in the US.

• #20 Undifferentiated Pleomorphic Sarcoma – Market Insight, Epidemiology and Market Forecast – 2034

  https://www.researchandmarkets.com/reports/5524902/undifferentiated-pleomorphic-sarcoma-market?srsltid=AfmBOoqSmPbQcyZuC1JIeVNbrqWc-y1V5VN2RjYdohjkFbjQrh2eRZ3T

  The Undifferentiated Pleomorphic Sarcoma epidemiology division provide insights about historical and current Undifferentiated Pleomorphic Sarcoma patient pool and forecasted trend for every seven major countries. […] The disease epidemiology covered in the report provides historical as well as forecasted Undifferentiated Pleomorphic Sarcoma epidemiology scenario in the 7MM covering the United States, EU5 countries (Germany, Spain, Italy, France, and the United Kingdom), and Japan from 2020 to 2034. […] The epidemiology segment also provides the Undifferentiated Pleomorphic Sarcoma epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.

• #21 Epidemiology of musculoskeletal tumours in a reference unit | Revista Española de Cirugía Ortopédica y Traumatología

  https://www.elsevier.es/es-revista-revista-espanola-cirugia-ortopedica-traumatologia-129-resumen-epidemiology-musculoskeletal-tumours-in-S1888441524002066

  Sarcoma and musculoskeletal tumours should be treated in referral centres to improve their multidisciplinary management. […] One of the reasons for the poorer prognosis of these patients is the late diagnosis of these tumours, and therefore knowing their epidemiology allows us to guide the distribution of resources and optimise their diagnosis and referral to referral centres. […] In conclusion, our epidemiological data agree in most aspects with those published in studies carried out in other areas. However, multicentre studies at a national level would be very useful to understand the epidemiology of these tumours in our country, and to be able to adapt resources to optimise their treatment.

• #21 Epidemiology of musculoskeletal tumours in a reference unit | Revista Española de Cirugía Ortopédica y Traumatología

  https://www.elsevier.es/es-revista-revista-espanola-cirugia-ortopedica-traumatologia-129-resumen-epidemiology-musculoskeletal-tumours-in-S1888441524002066

  Epidemiology of musculoskeletal tumours in a reference unit […] The objective of this study was to perform an epidemiological analysis of patients presented to the Musculoskeletal Tumors Committee of a reference hospital. […] A total of 1978 patients were analysed, of which 1477 (74.67%) were diagnosed as sarcomas. […] Our study represents the first work on the epidemiology of sarcomas and other musculoskeletal tumours in our country, being very useful to adapt the resources destined for their diagnosis and treatment. […] Knowledge of the epidemiological characteristics and geographical distribution of these tumours is very important for appropriate clinical suspicion and for optimising the referral of these patients to specialist units. […] This study was approved by the Ethics Committee of our institution, whose registration number is 2023-752-1.

• #22 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  The accurate staging of UPS is essential for optimal treatment planning and prognostication. […] The prognosis of UPS varies significantly depending on several key factors. […] A deep tumor location and AJCC stage are among the most critical prognostic factors for UPS, predicting both the potential for disease recurrence and overall survival. […] Collaborative research and data-sharing are vital in advancing our understanding of retroperitoneal UPS and developing effective treatments.

• #23 Undifferentiated Pleomorphic Sarcoma Market Outlook and Forecast – Thelansis

  https://thelansis.com/reports/undifferentiated-pleomorphic-sarcoma-market-outlook-forecast/

  The SEER program reports that among 26,758 cases, 17.1% were UPS, making it the second most frequent STS after leiomyosarcoma, regardless of the primary tumor location. Males have a significantly higher incidence rate than females. […] KOLs across 8 MM markets from the center of Excellence/ Public/ Private hospitals participated in the study. Insights around current treatment landscape, epidemiology, clinical characteristics, future treatment paradigm, and Unmet needs. […] This report presents the most important clinical unmet needs in the treatment, according to Thelansis research and analysis. Other essential unmet needs identified through our study include decreased cost burden on patients, improved administration convenience, and improved patient compliance.

• #24 Frontiers | Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2017.00091/full

  HTLV-1 is distributed worldwide, with areas of high endemicity close to others without the presence of infection. […] Approximately 10 million individuals are infected with HTLV-1 worldwide, and of them, 1–5% will develop an associated pathology. […] HTLV-1 is a human retrovirus capable of causing cancer, and it has been recently classified as group 1 human carcinogens by The IARC. […] In this case, sexual transmission from her husband was confirmed, who got infected from her mother who died of ATLL years before in a non-endemic area of Argentina. […] There are no cases reported in the literature of UPS linked to HTLV-1 infection. […] In this case, the patient had no laboratory results or symptoms related to an immunocompromised status even though it should not be discarded due to HTLV-1 infection which could contribute to UPS oncogenesis by both, direct and indirect mechanisms. […] Given the high grade of UPS and the progressive invalidating myelopathy caused by HTLV-1, the treatment should be carefully evaluated to positively impact on the patient’s life expectancy.

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