Malignant Soft Tissue Tumors Research Articles

Endometriosis of a postoperative scar

One of the long-term complications of abdominal surgery is endometriosis of the postoperative scar (EPS). The frequency of this condition is 0.03–1.08%. Mechanical implantation of endometrium during primary surgery plays a leading role in the pathogenesis of EPS. Together with clinical manifestations, ultrasound and magnetic resonance imaging are important diagnostic tools for this postoperative complication. Differential diagnosis requires excluding suture granulomas, hematomas, fibroids, hernias, lipomas, seromas, abscesses, and other malignant and benign soft tissue tumors in the area of the scar. This article describes a case of EPS in two women who had undergone cesarean section. Adequate removal of endometrial infiltrates, followed by abdominoplasty, allowed them to avoid recurrence and achieve optimal cosmetic results. Currently, in order to prevent the occurrence of EPS after surgical delivery, it is recommended to change gloves after removing the afterbirth and before restoring the integrity of the anterior abdominal wall, as well as limiting the edges of the wound to the abdominal cavity.

Development of an Artificial Intelligence System for Distinguishing Malignant from Benign Soft Tissue Tumors Using Contrast-Enhanced MR Images.

The integration of artificial intelligence (AI) into orthopedics has enhanced the diagnosis of various conditions; however, its use in diagnosing soft-tissue tumors remains limited owing to its complexity. This study aimed to develop and assess an AI-driven diagnostic support system for magnetic resonance imaging (MRI)-based soft-tissue tumor diagnosis, potentially improving accuracy and aiding radiologists and orthopedic surgeons. Experienced orthopedic oncologists and radiologists annotated 720 images from 77 cases (41 benign and 36 malignant soft-tissue tumors). Eleven tumor subtypes were identified and classified into benign and malignant groups based on histological diagnosis. Utilizing the standard machine learning classifier pipeline, we examined and down-selected imaging protocols and their predominant radiomic features within the tumor's three-dimensional region to differentiate between benign and malignant tumors. Among the scan protocols, contrast-enhanced T1 weighted fat-suppressed images showed the most accurate classification based on radiomics features. We focused on the two-dimensional features from the largest tumor boundary surface and its neighboring slices, leveraging texture-based radiomic and deep convolutional neural network features from a pretrained VGG19 model. The test data comprised 44 contrast-enhanced images (22 benign and 22 malignant soft-tissue tumors) containing six malignant and five benign subtypes distinct from the training data. We compared expert and non-expert human performances against AI by assessing malignancy detection and the time required for classification. The AI model showed comparable accuracy (AUC 0.91) to that of radiologists (AUC 0.83) and orthopedic surgeons (AUC 0.73). Notably, the AI model processed data approximately 400 times faster than its human counterparts, showcasing its capacity to significantly boost diagnostic efficiency. We developed an AI-driven diagnostic support system for MRI-based soft-tissue tumor diagnosis. While additional refinement is necessary for clinical applications, our system has exhibited promising potential in differentiating between benign and malignant soft-tissue tumors based on MRI.

An aggressive gastric CIC-DUX4 sarcoma surgically resected with multivisceral organs: a case report

BackgroundCapicua transcriptional repressor-double homeobox 4 sarcoma (CDS) is a rare and aggressive malignant soft tissue tumor that typically arises within the soft tissues. We report an exceptionally rare case of a gastric CDS successfully resected despite its extensive invasion into surrounding organs.Case presentationA 48-year-old male presented with a progressively enlarging abdominal mass. Upper gastrointestinal endoscopy revealed a large ulcerative tumor on the posterior gastric wall. Biopsy results initially suggested a neuroendocrine cell carcinoma. Contrast-enhanced computed tomography showed a 20 cm tumor protruding from the posterior stomach wall, directly invading the pancreas and colon. We performed a multivisceral resection (stomach, pancreatic tail, spleen, and transverse colon) achieving an R0 resection. Pathological examination of the permanent specimen revealed small round cells with high nuclear-to-cytoplasmic ratios. Immunohistochemical staining confirmed the diagnosis of CDS. The patient recovered well and was discharged on postoperative day 33.ConclusionsThis case report describes the first detailed account of a surgically resected aggressive CDS originating from the stomach.

SOFT TISSUE TUMORS OF THE LOWER LEG, FOOT AND ANKLE: A CROSS-SECTIONAL OBSERVATIONAL STUDY ANALYSING 376 CASES

Fewer than 5 % of soft tissue sarcomas arise in the foot and ankle. It can be difficult to distinguish between benign and malignant lesions which leads to a delay in diagnosis. Initial inappropriate procedures limit options for limb salvage and increasing rates of local recurrence. Our aim is to improve understanding of the presentation and management of these rare tumors to reduce delays in diagnosis and decrease the occurrence of inappropriate or unwarranted procedures. A prospectively maintained database of 376 new referrals to the West of Scotland regional musculoskeletal oncology service for soft tissue lesions of the foot, ankle, and lower leg over a 10-year period was analysed retrospectively. An assessment was made of patient demographics, presentation, anatomical location, diagnosis, classification, management, and outcomes for all patients. Of all new referrals, 53.5 % were diagnosed with primary benign soft tissue tumors and 16 % with primary malignant soft tissue tumors. The most common primary benign tumor in our population was schwannoma (15.9 %) and primary malignant tumor was undifferentiated sarcoma (26.7 %). In the foot alone, soft tissue sarcomas most commonly occurred in the forefoot (44.4 %). The most common presenting complaints were rest pain and focal swelling. Symptoms were present for on average 7 months prior to referral. Death from disease in sarcomas was 41.7 % over a 10 year follow up period, higher than other body areas. Soft tissue sarcomas in the foot and ankle remain a diagnostic challenge. Local biopsies should only be performed following discussion with an oncology surgeon. We have provided a management protocol in order to reduce the number of inappropriate procedures performed in this group and expedite referral to specialist centres, optimising clinical outcomes and reducing the cost of litigation to healthcare services.

Rare presentation of primary intra-articular synovial sarcoma of knee joint with insights from literature review

ABSTRACT Synovial sarcoma is a rare and malignant soft tissue tumor, comprising 5%–10% of such cases. This tumor primarily afflicts adolescents and young adults, with slight male bias, often near large joints like the knee and hip. Intra-articular synovial sarcoma, an even rarer subset, can delay diagnosis due to its slow growth and vague clinical signs. We present a unique case of primary knee intra-articular synovial sarcoma in a 24-year-old female. Imaging revealed a distinct lesion adjacent to the medial femoral condyle and patellofemoral joint, initially suggestive of synovial haemangioma. However, histopathology confirmed biphasic synovial sarcoma, characterized by spindle and gland-like epithelial components. Immunohistochemistry further supported the diagnosis. Reviewing documented cases, we find that synovial sarcoma can affect a wide age range but often occurs in the knee joint, emphasizing the need for vigilance. Early detection and intervention are crucial for improved treatment outcomes in this rare but challenging malignancy.

A rare case report of paratesticular leiomyosarcoma

A leiomyosarcoma (LMS) is a malignant mesenchymal tumor of soft tissue that rarely presents in the scrotal region. This case report describes a primary paratesticular LMS originating in the dartos layer of the scrotum in a symptomatic 37-year-old male with a history of hypertension, generalized seizure disorder post-excision of meningioma, and an extensive family history of cancer. Primary paratesticular LMSs in the superficial dartos layer are exceedingly rare. Given the rarity of this particular type of LMS, this case report includes a discussion on its prevalence and incidence. The rarity of this tumor also warrants a review of the literature and related cases to inform the treatment plan and enhance understanding of these oncologic conditions.

Consensus recommendations for systemic therapies in the management of relapsed Ewing sarcoma: A report from the National Ewing Sarcoma Tumor Board.

Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue that most often occurs in children, adolescents, and young adults. Debate and controversy remain in the management of relapsed/refractory ES (RR-ES). The authors leveraged the expertise assembled by the National Ewing Sarcoma Tumor Board, a multidisciplinary virtual tumor board that meets monthly to discuss challenging cases of ES. In this review, they focus on select topics that apply to the management of patients with RR-ES. The specific topics covered include the initial approach of such patients and discussion of the goals of care, the role of molecular testing, chemotherapy regimens and novel agents to consider, the role of maintenance therapy, and the use of high-dose chemotherapy with autologous stem cell rescue. The data referenced are often limited to subgroup analyses and/or compiled from multiple sources. Although not intended to replace the clinical judgement of treating physicians, these guidelines are intended to support clinicians and provide some clarity and recommendations for the management of patients with RR-ES. PLAIN LANGUAGE SUMMARY: Ewing sarcoma (ES) is a bone and soft tissue cancer that most often occurs in teenagers and young adults. This article uses the experience of the National Ewing Sarcoma Tumor Board, a multi-institution, multidisciplinary virtual tumor board that meets monthly to discuss challenging cases of ES and to address questions related to the treatment of patients with relapsed ES. Although not intended to replace the clinical judgement of treating physicians and limited by available data, these consensus recommendations will support clinicians who treat patients with this challenging malignancy, made even more difficult when it recurs.

Giant mucinous liposarcoma of the abdominal cavity: A case report.

Mucinous liposarcoma myxoid liposarcoma is a malignant mucoid soft tissue tumor derived from undifferentiated stromal cells in perivascular, subbody cavity and intermuscular space, and composed of cells at different stages of differentiation from preadipocytes to mature cells. In rare cases, it may change from lipoma malignancy. The main manifestations is painless mass, relatively slow growth, the course can last decades, the prevalence of liposarcoma in the population is 14% to 18%, mainly in adults, male prevalence is higher than women, but not significant. The main good hair part is the thigh, have mucinous sex, high differentiation type, dedifferentiation type, polymorphic type. Clinical diagnosis is difficult, and there are no obvious symptoms in the early stage, so the diagnosis should be combined with B ultrasound, MRI, CT, and other auxiliary examinations. The gold standard is pathological examination. In December 2023, our department admitted a patient with a mucinous abdominal mass. The report is as follows. Does liposarcoma metastasize? Is any chemotherapy required after surgery? Will it ever relapse in the future? What is the survival period after surgery? Mucinous liposarcoma. Surgical resection of the sarcoma. The nodule sample was 33 * 28 * 13 cm, with complete capsule, gray and yellow sections, fine texture, soft, gray, red, grayish, and yellow mucoid nodules in some areas, and the maximum diameter of the nodules was 21cm. Immunohistochemistry was: CD34 (+), CDK 4 (+), CK (-), Desmin (weak +), Ki67 (index 5%), MDM 2 (-), p16 (weak +), S-100P (+), Vimentin (+), BCL-2 (+). He was also sent to the Department of Pathology of Peking Union Medical College Hospital for consultation with Professor Lu Zhaohui, whose consultation opinion was in line with myxoliposarcoma. Retroperitoneal liposarcoma is a common retroperitoneal tumor, but it is relatively rare in clinical practice; the overall morbidity is low, mainly manifested as abdominal pain and abdominal distension, abdominal distension, and a long course of disease; it is not sensitive to radiotherapy and chemotherapy, and should be closely follow up by CT examination to understand the recurrence and metastasis.

Massive Bone Marrow Infiltration by Disseminated Alveolar Rhabdomyosarcoma Mimicking Acute Leukemia.

RMS is a malignant tumor of soft tissues affecting primarily children and adolescents. Around 6% to 23% RMS patients present bone marrow infiltration but leukemia-like involvement is very rare; in these patients cytomorphology on bone marrow smears can lead to misdiagnosis. Differential diagnosis with alveolar RMS should be kept in mind in every pediatric patient presenting with a marked bone marrow involvement in the absence of typical lymphoproliferative findings.

Does adding sacroiliac (type IV) resection to periacetabular (type II) resection increase complications or provide worse clinical outcomes? An institutional experience and systematic review

Background and objectivesInternal hemipelvectomy is a limb sparing procedure most commonly indicated for malignant bone and soft tissue tumors of the pelvis. Partial resection and pelvic reconstruction may be challenging for orthopedic oncologists due to late presentation, high tumor burden, and complex anatomy. Specifically, wide resection of tumors involving the periacetabular and sacroiliac (SI) regions may compromise adjacent vital neurovascular structures, impair wound healing, or limit functional recovery. We aimed to present a series of patients treated at our institution who underwent periacetabular internal hemipelvectomy (Type II) with or without sacral extension (Type IV) in combination with a systematic review to investigate postoperative complications, functional outcomes, and implant and patient survival following pelvic tumor resection via Type II hemipelvectomy with or without Type IV resection. Materials and methodsA surgical registry of consecutive patients treated with internal hemipelvectomy for primary or secondary pelvic bone tumors at our institution since 1994 was retrospectively reviewed. All type II resection patients were stratified into two separate cohorts, based on whether or not periacetabular resection was extended beyond the SI joint to include the sacrum (Type IV), as per the Enneking and Dunham classification. Patient demographics, operative parameters, complications, and oncological outcomes were collected. Categorical and continuous variables were compared with Pearson's chi square or Fisher's exact test and the Mann-Whitney U test, respectively. Literature review according to PRISMA guidelines queried studies pertaining to patient outcomes following periacetabular internal hemipelvectomy. The search strategy included combinations of the key words “internal hemipelvectomy”, “pelvic reconstruction”, “pelvic tumor”, and “limb salvage”. Pooled data was compared using Pearson's chi square. Statistical significance was established as p < 0.05. ResultsA total of 76 patients were treated at our institution with internal hemipelvectomy for pelvic tumor resection, of whom 21 had periacetabular resection. Fifteen patients underwent Type II resection without Type IV involvement, whereas six patients had combined Type II/IV resection. There were no significant differences between groups in operative time, blood loss, complications, local recurrence, postoperative metastasis, or disease mortality. Systematic review yielded 69 studies comprising 929 patients who underwent internal hemipelvectomy with acetabular resection. Of these, 906 (97.5 %) had only Type II resection while 23 (2.5 %) had concomitant Type II/IV resection. While overall complication rates were comparable, Type II resection alone produced significantly fewer neurological complications when compared to Type II resection with sacral extension (3.9 % vs. 17.4 %, p = 0.001). No significant differences were found between rates of wound complications, infections, or construct failures. Local recurrence, postoperative metastasis, and survival outcomes were similar. Type II internal hemipelvectomy without Type IV resection on average produced higher postoperative MSTS functional scores than with Type IV resection. ConclusionIn our series, the two groups exhibited no differences. From the systematic review, operative parameters, local recurrence or systemic metastasis, implant survival, and disease mortality were comparable in patients undergoing Type II internal hemipelvectomy alone compared to patients undergoing some combination of Type II/IV resection. However, compound resections increased the risk of neurological complications and experienced poorer MSTS functional scores.

Importance and implementation of radiological modalities in the diagnostics of soft tissue tumors

Malignant soft tissue tumors, in particular, require amultimodal treatment concept involving interdisciplinary cooperation between radiologists, pathologists, surgeons and oncologists at special tumor centers. The foundations of the treatment decision are the imaging diagnostics and the diagnosis confirmation based on tissue samples. The (local) extent and growth behavior of atumor are among the most important findings of imaging as they have adirect influence on the surgical procedure. The most important diagnostic procedure here is magnetic resonance imaging (MRI). The T1-weighted and fat-suppressed sequences after i.v. contrast administration are used to visualize the extent of the tumor. In synopsis with diffusion-weighted and T2-weighted sequences, adifferentiation between vital tumor tissue and tumor necrosis is additionally possible. This also enables targeted sampling from vital tumor parts so that the patient can be assigned to the appropriate treatment concept as quickly as possible.

Laryngeal nodular fasciitis in a 75-year-old man: a rare case report and review of the literature.

Nodular fasciitis (NF) is a benign and self-limiting fibroblastic proliferation that originates from the superficial fascia and extends into the subcutaneous tissue or muscle. It typically manifests in individuals aged 20 to 35 years, with rare occurrences observed in patients over the age of 60 years. We herein report a case involving a 75-year-old man with NF in the right vocal cord. The patient sought medical attention at the Department of Otolaryngology of our hospital because of a 1-month history of hoarseness and breathlessness. The diagnosis was unable to be confirmed through preoperative pathological examination. After admission to our hospital, various examinations were completed and surgical treatment was performed, and the postoperative histopathological findings revealed the presence of NF in the right vocal cord. NF of the vocal cord is a rare clinical entity. Given its rapid progression and propensity for marked infiltration, it often poses diagnostic challenges because it can mimic various malignant soft tissue tumors. Therefore, thorough exclusion of other neoplastic lesions is imperative prior to confirming the diagnosis of NF through pathological examination. Local surgical resection remains the primary treatment modality.

Innovations in the classification of soft tissue tumors

Soft tissue tumors are avery heterogeneous group of tumors. Their classification is regularly updated by the World Health Organization (WHO), most recently in 2020. The current classification of soft tissue tumors emphasizes molecular biological tumor characteristics, which enable tumor-specific treatment. In addition to Ewing's sarcoma, which occurs as bone as well as extra-skeletal soft tissue tumors as asmall round cell sarcoma, three other subtypes of undifferentiated, small and round cell sarcomas have been introduced. Some names of the new sarcomas can be derived from the gene mutations. The groups of adipocytic and (myo)fibroblastic tumors have been extended by three further entities. There were further additions to vascular soft tissue tumors, smooth muscle cell tumors, peripheral nerve sheath tumors and tumors of uncertain differentiation. Adistinction is made between benign, intermediate locally aggressive, intermediate rarely metastatic and malignant soft tissue tumors.

Histological diagnostic discrepancy and its clinical impact in bone and soft tissue tumors referred to a sarcoma center.

Histological diagnosis of sarcomas (malignant bone and soft tissue tumors) is challenging due to their rarity, morphological diversity, and constantly evolving diagnostic criteria. In this study, we aimed to assess the concordance in histological diagnosis of bone and soft tissue tumors between referring hospitals and a tertiary sarcoma center and analyzed the clinical impact of the diagnostic alteration. We analyzed 628 consecutively accessioned specimens from 624 patients who visited a specialized sarcoma center for treatment. The diagnoses at referring hospitals and those at the sarcoma center were compared and classified into four categories: agreed, disagreed, specified, and de-specified. Of the 628 specimens, the diagnoses agreed in 403 (64.2%) specimens, whereas some changes were made in 225 (35.8%) specimens: disagreed in 153 (24.3%), specified in 52 (8.3%), and de-specified in 20 (3.2%) cases. The benign/intermediate/malignant judgment changed for 92 cases (14.6%). The diagnostic change resulted in patient management modification in 91 cases (14.5%), including surgical and medical treatment changes. The main inferred reason for the diagnostic discrepancies was a different interpretation of morphological findings of the tumor, which accounted for 48.9% of the cases. This was followed by the unavailability of specialized immunohistochemical antibodies and the unavailability of genetic analysis. In summary, our study clarified the actual clinical impact of diagnostic discrepancy in bone and soft tissue tumors. This may underscore the value of pathology consultation, facilitating access to specialized diagnostic tools, and continued education. These measures are expected to improve diagnostic precision and ultimately benefit patients.

Molecular genetics and research progress of uterine leiomyosarcoma.

Uterine leiomyosarcoma (uLMS) is a type of malignant soft-tissue tumor, which is developed from myometrium in the female reproductive system. This disease is difficult to be distinguished from benign uterine leiomyoma in the early stages, but it progresses aggressively and relentlessly. Hence, uLMS has a dismal prognosis and high rates of both misdiagnosis and missed diagnosis. Unfortunately, current studies of uLMS pathogenesis and disease biology are inadequate. uLMS disease models are also very limited, hindering the development of effective therapeutics. In this review, we focus on the pathological molecular biology of uLMS, and systematically review the molecular genetic features, epigenetic variants, experimental models, and clinical research progress of uLMS. We further discuss the development direction and potential needs of uLMS in the fields of tumor evolution, tumor microenvironment, and tumor therapy, with the aim of providing a better understanding of the pathobiological mechanism of uLMS and providing a reference for the development of potential diagnostic and therapeutic strategies.

Incidence and patterns of lymph node metastases in head and neck rhabdomyosarcoma: One-institution study.

Head and neck rhabdomyosarcoma (HNRMS) is an aggressive malignant soft tissue tumor that easily develops lymph node metastasis (LNM) and distant metastasis. No literature investigates the pattern of LNM in HNRMS. Ninety-five consecutive patients with HNRMS newly diagnosed at one institution between November 2011 and July 2023 were retrospectively reviewed. All the patients underwent head and neck contrast-enhanced MRI and/or CT, PET-CT if necessary. The associations between LNMs and clinical characteristics and histopathological parameters were discovered. 44.2% of patients had evidence of LNM at diagnosis, and the most common LNM occurred in the ipsilateral retropharyngeal space. The primary tumor metastasizes to the retropharyngeal space, and then next to level II is the most common LN drainage basin. In multivariate analysis, only distant metastasis determines the prognosis, other than LN status. LNM has a high incidence in HNRMS and rarely causes contralateral metastasis for localized lesions or skip metastasis.

Neurofibromatosis to neoplasia transition: a rare case report of spindle cell malignant peripheral nerve sheath tumor with literature review

Introduction and Importance: Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue malignant tumor. MPNST in the spinal canal are rarely seen except in cases with NF1. But a long segment extradural spinal malignant spindle cell neoplasm has not been reported in current literature. Case Presentation: We present the first reported case of spinal malignant spindle cell neoplasm extended along the spine. The detected lesion is responsible for compressing various segments of the spinal cord, causing thinning of the cord and secondary stenosis of the spinal canal, leading to a condition known as multi-segment compression myelopathy. Clinical Discussion: MPNSTS are typically detected late due to nonspecific symptoms, with a higher incidence in extremities and a notable occurrence in unusual locations. Diagnosis relies on MRI and histopathology, with S_100 positivity as a neural marker. MPNSTs can arise from neurofibromas or Schwann cells, with a significant portion resulting from TP53 mutations or secondary to radiation exposure. Conclusion: This case stands out due to its unique presentation, characterized by a predominantly spindle cell morphology with certain epithelioid features. It is imperative to recognize this condition for an accurate diagnosis, emphasizing the spindle cell-type malignant peripheral nerve sheath tumor (MPNST) and highlighting its exceptionally poor prognosis.

Ameloblastic fibrosarcoma of the maxilla arising in an old woman, a rare case report and literature review

BackgroundAmeloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone.Case presentationA 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up.ConclusionBased on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

QOL-31. NEUROPSYCHOLOGICAL FUNCTIONING AND QUALITY OF LIFE IN INFANT AT/RT SURVIVORS: FOCUS ON FLUID INTELLIGENCE AND VISUAL PROCESSING

Abstract BACKGROUND Understanding the long-term cognitive sequelae in infant brain tumor survivors remains incomplete, particularly regarding the impact of tumor type, multimodal treatment, and other patient-related factors. This retrospective analysis explores neuropsychological and quality of survival (QoS) outcomes in survivors of atypical teratoid/rhabdoid tumors (AT/RT) and extracranial malignant rhabdoid tumors of soft tissues (eMRT) and kidneys (RTK), all treated within the same framework. Neuropsychological data from children with AT/RT were compared to data from children with non-irradiated low-grade glioma (LGG). METHODS Patients (0 - 36 months at diagnosis) underwent various treatments, including radio-chemotherapy for AT/RT (n = 13) and eMRT/RTK (n = 7), chemotherapy only for LGG (n = 4) and eMRT/RTK (n = 1), or observation for LGG (n = 11). Neuropsychological evaluations were conducted at a median of 6.8 years (AT/RT), 6.6 years (eMRT/RTK), and 5.2 years (LGG) post-diagnosis. RESULTS Impairments were observed for all tumour types. Patients with AT/RT exhibited impairments in fluid intelligence (p =.041; d = 1.11) and visual processing (p =.001; d = 2.09) when compared to LGG-patients. Both groups demonstrated deficits in psychomotor speed and attention abilities (p &amp;lt;.001–.019; d = 0.79–1.90). Diagnosis significantly predicted cognitive outcomes, whereas gender and age-related variables did not. QoS outcomes for all rhabdoid patients indicated lower scores in psychosocial functioning (p =.023; d = 0.78) and quality of life (p =.006; d = 0.79) compared to healthy controls. CONCLUSIONS Infant rhabdoid tumor survivors experience cognitive and quality-of-life sequelae. Patients with AT/RT are especially vulnerable to impairments in fluid intelligence and visual processing, while infant LGG-patients without radiotherapy demonstrated comparable deficits in psychomotor and attention abilities. Close monitoring of neuropsychological and quality of life outcomes is crucial for early onset and multimodal treatment.

Plexiform fibrohistiocytic tumor: A series of 10 case studies.

We sought to investigate the clinicopathologic features and differential diagnosis of plexiform fibrohistiocytic tumor (PFHT) and its pathogenesis. Ten cases of PFHT were collected from Xi Jing Hospital, Fourth Military Medical University, from September 2008 to December 2022 for clinical data as well as microscopic and immunohistochemical observation. CCND1 gene amplification and break were assayed by fluorescence in situ hybridization (FISH). We report 10 cases of PFHT according to histologic classification. Seven cases were of histiocytoid type, and 3 had mucous degeneration in the nodules. One case was of fibroblastic type, which was mainly composed of fibroblast-like cells. Two cases were of mixed type. Immunohistochemically, the osteoclast-like multinucleated giant cells, histiocyte-like cells, and occasional spindle cells in the adjacent fascicles were reactive for CD68 (10/10), CD163 (5/8), CD10 (8/8), cyclin D1 (8/8), CDK4 (5/8), β-catenin (4/6), MITF (2/6), and PGP9.5 (4/5). Vimentin (9/9) was strongly positive in tumor cells and peripheral fibroblast-like cells. The positive index of Ki-67 was 5% to 40%, with an average of 20%. The FISH analysis showed neither amplification nor break of the CCND1 gene. All cases underwent surgical resection, and patients were followed up for 9 months to 11 years. Only 2 cases recurred. Plexiform fibrohistiocytic tumor is a low-grade malignant soft tissue neoplasm. The diagnosis mainly depends on histopathologic and immunohistochemical markers. Cyclin D1 and CD10 expression has diagnostic value for the diagnosis and differential diagnosis of PFHT combined with its plexiform morphology. The overexpression of cyclin D1 suggests an involvement of cell cycle regulatory genes in the pathogenesis of PFHT.