Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Abstract
Bone marrow is thought to be a primary hematopoietic organ. However, accumulated evidences demonstrate that active function and trafficking of immune cells, including regulatory T cells, conventional T cells, B cells, dendritic cells, natural killer T (NKT) cells, neutrophils, myeloid-derived suppressor cells and mesenchymal stem cells, are observed in the bone marrow. Furthermore, bone marrow is a predetermined metastatic location for multiple human tumors. In this review, we discuss the immune network in the bone marrow. We suggest that bone marrow is an immune regulatory organ capable of fine tuning immunity and may be a potential therapeutic target for immunotherapy and immune vaccination.

https://www.nature.com/articles/cmi201147

I’d like to preface this post-
“Soft tissue tumors”

Overview
Tumor in thigh muscle
Soft tissue sarcoma Open pop-up dialog box
Soft tissue sarcoma is a rare type of cancer that begins in the tissues that connect, support and surround other body structures. This includes muscle, fat, blood vessels, nerves, tendons and the lining of your joints.

More than 50 subtypes of soft tissue sarcoma exist. Some types are more likely to affect children, while others affect mostly adults. These tumors can be difficult to diagnose because they may be mistaken for many other types of growths.

https://www.mayoclinic.org/diseases-con ... c-20377725

Synopsis

Pulmonary metastases are a common manifestation of sarcoma that requires special attention. For decades, surgical resection of pulmonary metastases has been performed, and despite limited randomized data, surgery is increasingly accepted as an integral part in the management of metastatic disease. Although sarcoma is heterogeneous, common prognostic factors have been identified to guide surgeons prior to pulmonary metastasectomy including long disease-free interval, ability to obtain a complete resection, and extra-pulmonary disease control. Patients with adequate pulmonary reserve may undergo resection via thoracotomy or in select cases video-assisted thoracoscopic surgery (VATS). Long-term results indicate resection is potentially curative with significantly improved survival following complete resection. Recurrence, however, is not uncommon with many patients undergoing repeat resection. With advancing surgical technique and adjuvant therapies, patients with high or recurrent tumor burden are increasingly afforded disease control and potential cure. In this review, the prognostic characteristics of pulmonary metastases from sarcoma, pre-operative evaluation, operative technique, long-term outcomes, and management of complex patients are highlighted.

Keywords: Sarcoma, pulmonary metastasectomy, thoracic surgery, prognostic factors, VATS, SBRT


https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5028117/

Abstract

Background
We surveyed the natural history of bone metastases in patients affected by soft tissue sarcoma (STS).

Methods
This multicenter retrospective observational study included 135 patients. Histological subtype, characteristics of bone metastases, treatment, skeletal related events (SREs) and disease outcome were recorded.

Results
The most represented histological subtypes were leiomyosarcoma (27%) angiosarcoma (13%) and undifferentiated sarcoma (8%). Axial skeleton was the most common site for bone involvement (70%). In 27% of cases, bone metastases were present at the time of diagnosis. Fifty-four (40%) patients developed SREs and the median time to first SRE was 4 months (range 1–9). The most common SRE was the need for radiotherapy (28%) followed by pathological fracture (22%). Median survival after bone progression was 6 months (range 1–14). SREs were associated with decreased overall survival (OS) (P = 0.04). A subgroup analysis revealed that bisphosphonates significantly prolonged median time to first SRE (5 versus 2 months; P = 0.002) while they did not determine an improvement in OS, although a favourable trend was identified (median: 7 versus 5 months; P = 0.105).

Conclusions
This study illustrates the burden of bone disease from STS and supports the use of bisphosphonates in this setting.



https://clinicalsarcomaresearch.biomedc ... 5-3329-3-6

Methotrexate-associated lymphoproliferative disorder complicated by bisphosphonate-related osteonecrosis of the jaw arising in a female rheumatoid arthritis patient: Report of a case☆



Abstract
Methotrexate-associated lymphoproliferative disorder (MTX-LPD) is a lymphoid proliferation or lymphoma in an immunosuppressed patient administered with MTX. Bisphosphonate-related osteonecrosis of the jaw (BRONJ) is a clinical condition characterized by bone exposure in the oromaxillofacial region of the patients administered with BPs. Here, we report a case of MTX-LPD complicated by BRONJ occurred in a 67-year-old Japanese woman with RA, who received MTX and alendronate sodium hydrate for several years. She had a mass with ulcer formation in the area of hard palate. Histopathologically, large atypical lymphoid cells were found to infiltrate into granulation tissue in the ulcerative oral mucosa. Immunohistochemistry showed that the large atypical lymphoid cells were positive for Ki-67, CD3, CD30, and CD79a and negative for CD15, ALK1, and AE1/AE3. Withdrawal of MTX therapy led to complete remission within 4 weeks. However, 9 weeks later, necrotic bone was formed on hard palate and sequestrectomy was performed twice. Thereafter, the operation for closing naso-oral fistula was performed. The patient has been free of recurrence for 1 year since fistula closure. This is a very rare case in which the oral cavity was the initial site of manifestation of MTX-LPD followed by BRONJ in the patient with RA.


https://www.sciencedirect.com/science/a ... 5814000088

Lymphoproliferative disorders (LPD)[1][2] comprise a heterogeneous group of diseases characterized by uncontrolled production of lymphocytes that cause monoclonal lymphocytosis, lymphadenopathy and bone marrow infiltration. These diseases often occur in immunocompromised individuals. There are two subsets of lymphocytes: T and B cells that regenerate uncontrollably to produce immunoproliferative disorders, which are prone to immunodeficiency, a dysfunctional immune system, and lymphocyte dysregulation. Several gene mutations have been described as causes of LPD that can be iatrogenic or acquired.

The X-linked LPD is characterized by a mutation in the X chromosome that predisposes to natural killer cell LPD and T-cell LPD. Autoimmune lymphoproliferative syndrome (ALPS) is a type of LPD caused by a mutation in the gene that encodes for a Fas protein which is located in the long arm of chromosome 10. Males with X-linked immunodeficiency syndrome are susceptible to LPD and at risk for acquiring EBV and further development of lymphoma.

Individuals with common variable immunodeficiency (CVID), severe combined immunodeficiency (SCID), Wiskott-Aldrich syndrome, ataxia-telangiectasia, Chediak–Higashi syndrome and viral infections including HIV, are also prone to LPD. Others at risk include patients undergoing tissue transplantation and the use of immunosuppressive drugs such as cyclosporin, sirolimus, and tacrolimus. Invasive fungal infections have also been linked to this pathology.[3]

Chronic lymphoproliferative disorders are immuno-morphologically and clinically heterogeneous. Common features of these processes include various immunophenotypes (T, B, and NK cells) and terminal deoxynucleotidyl transferase negativity. The B-cell lymphocytic disorders include B-cell chronic lymphocytic leukemia, B-cell prolymphocytic leukemia, non-Hodgkin lymphoma (including mantle cell lymphoma) in leukemic phase, hairy cell leukemia and splenic lymphoma with villous lymphocytes. The T-cell chronic lymphoproliferative disorders include Sezary syndrome, T-cell prolymphocytic leukemia, adult T-cell leukemia-lymphoma, and large granulated lymphocyte leukemia.

https://www.ncbi.nlm.nih.gov/books/NBK5 ... e-24605_s1_

From Cytomorphology to Molecular Pathology: Maximizing the Value of Cytology of Lymphoproliferative Disorders and Soft Tissue Tumors



Abstract
Objectives:
The field of cytopathology has been rapidly advancing in the era of molecular pathology and personalized medicine. On-site cytologic evaluation for adequacy and triaging specimens for small core biopsy or fine-needle aspiration (FNA) are often required. Cytopathologists face the challenge of how to best triage small specimens for diagnosis, molecular testing, and personalized treatment. Owing to its minimally invasive nature, FNA alone or combined with core biopsy for lymphoproliferative disorders and soft tissue tumors has gained popularity.
Methods:
Literature review and author’s institutional experience are used for this review article. This article will focus mainly on lymphoproliferative disorders and soft tissue tumors.
Results:
Evaluation combining cytomorphology, immunohistochemistry, and/or molecular pathology is often needed to accurately diagnose and classify lymphomas and soft tissue tumors. Many molecular tests have been performed on cytologic specimens, such as tests for BRAF and RET in thyroid FNA.
Conclusions:
Molecular pathology has been widely integrated into conventional cytopathology for diagnosing lymphoproliferative disorders and soft tissue tumors, and the diagnostic value of FNA on those tumors has increased significantly. Cytology will play a more important role in the era of personalized medicine,

https://academic.oup.com/ajcp/article/140/4/454/1760497

Cytopathology is a diagnostic technique that examines cells from various body sites to determine the cause or the nature of disease. The first cytopathology test developed was the Pap test, which has been widely utilized in the last 50 years for screening and diagnosing of cervical cancer and its precursors.
American Medical Association › cyt...


https://www.ama-assn.org/specialty/cytopathology