Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas with poor prognosis due to their high recurrence rates. The prevalence of MPNST in the general population is 0.001%, with tumors arising from the retroperitoneum accounting for only 1% of all MPNSTs. In this report, we present a case of a 59-year-old male with pancreatic MPNST. To the authors’ knowledge, this is the first documented case of pancreatic MPNST in the Philippines.

The patient initially presented with a 3-month history of abdominal pain, weight loss, and anorexia. On abdominal computed tomography (CT) scan, a large cystic mass involving the pancreatic head and body, with an enhancing peripheral solid component in the superior region was seen. The patient underwent distal pancreatectomy, en bloc splenectomy and excision of duodenal cyst. Post-operative histopathology and immunohistochemistry staining were consistent with pancreatic MPNST with tumor very near the margin of resection adjacent to the portal vein. Adjuvant systemic chemotherapy and radiotherapy were not performed due to lack of evidence of benefit over risk for this population. Disease recurrence (nodal-peritoneal metastases) was noted six months post-operatively and he was given palliative chemotherapy with single-agent doxorubicin. However, disease progression was noted after five cycles of chemotherapy. Second-line regimen was planned but the patient died of a pulmonary embolism prior to the initiation of chemotherapy.

Due to the rarity and highly aggressive nature of MPNSTs, furthering knowledge on these tumors is important, particularly in their inclusion among the differential diagnoses for pancreatic tumors. Prompt diagnosis and histopathologic confirmation by a pathologist specializing in sarcomas are crucial in the treatment planning and prognostication of these tumors. Lastly, further studies are needed to establish more effective treatments in unresectable or metastatic disease.

Abstract Embryonal rhabdomyosarcoma (ERMS) is a primitive and aggressive soft tissue tumor that arises from premature mesenchymal cells and accounts for less than 1% of prostate malignancies. We present the case of a 26-year-old Filipino male who initially sought consultation and work-up for acute urinary retention. Although his prostate-specific antigen (PSA) level was normal, a kidney-ureter-bladder (KUB) ultrasound revealed an incidental finding of an enlarged prostate gland. Subsequent imaging tests (MRI and CT) identified a large, partially exophytic mass on the prostate with intravesical extension. The patient underwent a biopsy via transurethral resection at another institution, which yielded findings of a poorly differentiated carcinoma, primarily suggesting prostatic adenocarcinoma with a Gleason score of 10 (5+5). A request for slide review was submitted to our institution, and subsequent immunohistochemistry (IHC) studies—highlighting the tumor's immunoreactivity to myogenin and desmin—confirmed the diagnosis of ERMS. In this report, we discuss the clinical features, pathogenesis, treatment, diagnosis, and prognosis of this rare prostate tumor.
Embryonal rhabdomyosarcoma ; Immunohistochemistry ; Prostate ; Young adult

This study aims to establish the S_(180) tumor-bearing mice model, and to investigate the influence of Shenqi Erpi Granules(SQEPG) on immune function, as well as the drug's tumor-suppressive effect and mechanism. SPF grade KM mice(half male and half female) were randomly divided into 6 groups: a control group, a model group, a cyclophosphamide group(50 mg·kg~(-1)), as well as SQEPG groups in low-, medium-, and high-dose(5.25, 10.5, 21 g·kg~(-1)). The control group and the model group were given distilled water, and the other 4 groups were given the corresponding drugs by gavage. The administration continued for 10 days before the mice were sacrificed. The antitumor and immune regulation effects of SQEPG were evaluated. The effect of SQEPG on delayed type hypersensitivity reaction(DTH), carbon clearance index, and serum hemolysin antibody level was observed to reflect the effect on the immune function of tumor-bearing mice. Tumor weight was recorded to calculate the tumor suppression rate and the immune organ index. Hematoxylin-eosin(HE) staining was used to detect morphological changes in tumor tissues. Flow cytometry was employed to detect the percentage of CD4~+ and CD8~+ T-cells in the spleen tissues and the tumor tissue apoptosis levels. Immunohistochemistry was conducted to detect the KI67 protein expression level of tumor tissues. ELISA resorted to the detection of the following expression levels in tumor tissues: tumor necrosis factor-α(TNF-α), interleukin-2(IL-2), interferon-γ(IFN-γ). Western blot was performed to detect the expression levels of caspase-3, B-cell lymphoma-2(Bcl-2), Bcl-2-associated X protein(Bax), cyclin-dependent kinases 4(CDK4), G_1/S-specific cyclin D1(cyclin D1), and vascular endothelial growth factor A(VEGFA). The results showed that, compared with the model group, the SQEPG could increase the swelling of the auricle of the tumor-bearing mice; significantly increase the phagocytic index of carbon granule contour(P<0.05 or P<0.01), and the middle dose of SQEPG could significantly increase the antibody level of hemolysin(P<0.05); different doses of SQEPG significantly inhibit the growth of the tumor, and decrease the mass of the tumor tissues(P<0.05 or P<0.01); the low dose of SQEPG significantly decreased spleen index(P<0.05), low and high doses of SQEPG increased thymus index, while medium doses of SQEPG decreased thymus index. High doses of SQEPG significantly elevated the levels of CD4~+ and CD8~+ T-cells in the spleens of the homozygous mice(P<0.01 or P<0.001), and increased the apoptosis rate of the cells of the tumor tissues(P<0.05); Meanwhile, high-dose SQEPG elevated the levels of immunity factors such as IL-2, IFN-γ and TNF-α in the serum of tumor-bearing mice(P<0.01); medium-and high-dose SQEPG significantly lowered the rate of positive expression of KI67 protein in tumor tissues(P<0.01). Compared with the model group, high-dose SQEPG significantly up-regulated the expression of caspase-3 and Bax proteins in tumor tissues(P<0.05), and significantly down-regulated the expression of CDK4, cyclin D1, and VEGFA proteins(P<0.05 or P<0.01). In conclusion, SQEPG has the effect of improving immune function and inhibiting tumor growth in tumor-bearing mice. Its mechanism of tumor-suppressive effects may be related to apoptosis promotion, cell cycle progression block, and tumor cell proliferation inhibition.
Animals ; Mice ; Drugs, Chinese Herbal/pharmacology* ; Male ; Female ; Apoptosis/drug effects* ; Sarcoma 180/genetics* ; Humans

Primary malignant bone tumors are extremely rare. Osteosarcoma, chondrosarcoma, Ewing's sarcoma, and myeloma are the most common malignancy in bone. Osteosarcoma and Ewing's sarcoma are common in children and adolescents, and the tumors are high lethality due to the high rate of pulmonary metastasis. While chondrosarcoma, myeloma, and chordoma are more common in middle aged and elderly people. Japanese Orthopaedic Association (JOA) published the secondary clinical practice guideline on the management of primary malignant bone tumors. We put an emphasis on explanation some important issue of this guideline for help Chinese musculoskeletal tumor professionals in clinical practice.
Humans ; Bone Neoplasms/surgery* ; Chondrosarcoma/therapy* ; Japan ; Orthopedics ; Osteosarcoma/pathology* ; Practice Guidelines as Topic ; Sarcoma, Ewing/therapy* ; Societies, Medical

OBJECTIVES: This study aimed to evaluate the diagnostic accuracy of Cyclin D1 as an adjunct immunomarker in CD99 positive small round cell neoplasms with primary consideration of PNET/EWS. MATERIALS AND METHODS: Tissue from 2017 to 2023 with a histopathologic diagnosis of CD99 positive small round blue cell tumors with primary consideration of Primitive Neuroectodermal Tumor (PNET)/Ewing Sarcoma were retrieved and Cyclin D1 immunohistochemical staining done. Diagnostic accuracy of Cyclin D1 immunostaining was determined by calculating the sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: Cyclin D1 immunohistochemical staining was performed in 19 specimens available, of which 13 yielded a positive result. Of these, 8 had a final histopathologic diagnosis of CD99 positive small round blue cell tumor with primary consideration of PNET/Ewing Sarcoma, resulting in sensitivity of 61.54%, specificity of 100%, positive predictive value of 100% and negative predictive value of 50.0%. The overall accuracy is 72.2%. CONCLUSION Cyclin D1 can be used as an adjunct immunomarker to aid in the diagnosis of CD99 positive round cell tumor with primary consideration of PNET/Ewing Sarcoma specifically in resource limited settings where molecular testing is not readily available. Given the high specificity of Cyclin D1 in such cases, it can be used to rule out other small round blue cell tumors that can also stain positive for CD99 such as Rhabdomyosarcoma. However, interpretation must be done in conjunction with the results of other immunohistochemical stains in order to increase its diagnostic accuracy.
Human ; Male,Female ; Cells ; Sarcoma, Ewing ; Sarcoma ; Neuroectodermal tumors, Primitive ; Cyclin D1

Ewing’s sarcoma is a rare cancerous tumor of bone or soft tissue that usually occurs mostly in young adults. The diagnosis of Ewing’s sarcoma in pregnancy, most especially the subtype extraskeletal Ewing’s Sarcoma, is very rare with only few cases published in the literature worldwide. We present a case of a primigravida diagnosed with extraskeletal Ewing’s sarcoma at 6 weeks age of gestation. Currently, because of the rarity of this condition, there is lack of a universal consensus on the recommended therapeutic approach. A multidisciplinary management involving the generalist obstetrician, perinatologist, medical oncologist, and neonatologist was initiated at the outset to provide timely balance between optimal maternal treatment and fetal well-being. The maternal and fetal condition was stable all throughout the course of the chemotherapy using doxorubicin during pregnancy. Close interdisciplinary coordination regarding the treatment plans across these subspecialists resulted in a successful pregnancy outcome.

Embryonal rhabdomyosarcoma of the uterus is a rare condition with only a few cases documented. Exceedingly rare, however, is its concomitant incidence with uterine inversion. The infrequency with which genital tract sarcoma with uterine inversion is encountered makes the diagnosis and management a formidable challenge. The present case reports a 12-year-old nulligravida who complained of a rapidly growing introital mass of 3-month duration. Suspicion of nonpuerperal uterine inversion was confirmed by imaging, and malignancy was proven through adequate tissue sampling. While there is no unified protocol in the management of prolapsed genital tract sarcomas, the complete inversion of the corpus necessitated surgery. In the case presented, exploratory laparotomy and total hysterectomy through a double setup, abdomino-vaginal approach was done. The case illustrates the diagnostic, therapeutic, and ethical dilemmas in handling an aggressive tumor in an adolescent. Early recognition and a multidisciplinary approach are extremely crucial in ensuring improved prognosis and holistic treatment.

Myeloid sarcoma is a tumor that demonstrates extramedullary proliferation of myeloid blasts with or without maturation. It may present as an isolated tumor or may have peripheral or marrow involvement. The diagnosis of myeloid sarcoma is highly challenging as it may mimic other tumors.

A 71-year-old woman with an Eastern Cooperative Oncology Group (ECOG) performance score of 2 presented with a progressively enlarging right facial mass that had been growing for 18 months. Initially, it appeared as a 1x1 cm erythematous pustular lesion. A core biopsy suggested carcinoma, but COVID-19 delayed immunohistochemical (IHC) testing.

As the mass grew, eventually covering more than half of her face, a CT scan revealed a large, multilobulated mass involving the periorbital areas, nose, and upper lip. A repeat biopsy showed atypical round cell proliferation, and immunohistochemical staining confirmed myeloid sarcoma with CD34 and CD117 positivity. Bone marrow aspiration and biopsy ruled out leukemia.

The diagnosis of non-leukemic myeloid sarcoma was established. The patient was referred to plastic surgery, ophthalmology, and otorhinolaryngology for co-management of the mass. Initial treatment began with azacitidine, a hypomethylating agent. However, after completing only one cycle of chemotherapy, she declined further treatment for personal reasons, choosing not to continue with the planned therapeutic regimen.

Non-leukemic myeloid sarcoma of the face in an elderly patient is rare. Diagnosis was confirmed via biopsy and immunohistochemical studies. Treatment with azacitidine was chosen based on the patient’s ECOG score of 2. However, there is no consensus on its management, and the role of systemic chemotherapy remains debated. Continuous monitoring for progression to acute myeloid leukemia (AML) is crucial, as early detection significantly impacts prognosis and informs treatment decisions.

Extraskeletal Ewing's sarcoma（EES） in the head and neck is extremely rare, with non-specific clinical manifestations, high malignancy, easy recurrence and metastasis, and poor prognosis. This paper reports a case of EES of the parapharyngeal skull base presenting with secretory otitis media as the initial symptom.The treatment consisted of surgery, chemotherapy and radiotherapy. No further metastasis or recurrence was observed during the two years and six months follow-up. Now we reviewed the relevant literatures and summarized the experience of diagnosis and treatment in EES.
Humans ; Otitis Media with Effusion/etiology* ; Sarcoma, Ewing/therapy* ; Skull Base ; Skull Base Neoplasms/therapy*

Osteosarcoma (OS), chondrosarcoma (CS), and Ewing sarcoma (ES) represent primary malignant bone tumors and pose significant challenges in oncology research and clinical management. Conventional research methods, such as two-dimensional (2D) cultured tumor cells and animal models, have limitations in recapitulating the complex tumor microenvironment (TME) and often fail to translate into effective clinical treatments. The advancement of three-dimensional (3D) culture technology has revolutionized the field by enabling the development of in vitro constructed bone tumor models that closely mimic the in vivo TME. These models provide powerful tools for investigating tumor biology, assessing therapeutic responses, and advancing personalized medicine. This comprehensive review summarizes the recent advancements in research on 3D tumor models constructed in vitro for OS, CS, and ES. We discuss the various techniques employed in model construction, their applications, and the challenges and future directions in this field. The integration of advanced technologies and the incorporation of additional cell types hold promise for the development of more sophisticated and physiologically relevant models. As research in this field continues to evolve, we anticipate that these models will play an increasingly crucial role in unraveling the complexities of malignant bone tumors and accelerating the development of novel therapeutic strategies.
Bone Neoplasms/pathology* ; Humans ; Osteosarcoma/pathology* ; Tumor Microenvironment ; Sarcoma, Ewing/pathology* ; Chondrosarcoma/pathology* ; Animals ; Cell Culture Techniques/methods* ; Cell Culture Techniques, Three Dimensional/methods* ; Cell Line, Tumor