DOCSLIB.ORG

Case Report R

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Case Report R UNDIFFERENTIATED ROUND-CELL SARCOMA OF THE ILIUM (EWING TUMOR) CONTAINING HEMOPOIETIC ELEMENTS N. W. ROOME, M.D., AND P. A. DELANEY, Pu.D., M.D. (Prom the Departments of Surgerg and Pathology of the Universily oJ Chicago) The undifferentiated round-cell sarcomata of bone grouped together by Ewing (I) in 1922 as "diffuse endothelioma" are con- sidered an oncological entity by most authors. Considerable doubt exists, however, as to the origin of the Ewing sarcoma. Ewing and his supporters believe that it is derived from an endothelial cell of the bone marrow, possibly the perivascular lymphatic endothe- lium, and now apply the name "endothelial myeloma'' with that significance. Kolodny (2), Oberling (3), and others think that these tumors may arise from the reticulum cells or the reticulo- endothelium. Certain facts suggest a relation to the myelomata: multiple tumors frequently appear early in the disease, there is usually regression under radiotherapy, and the structure may "verge on myelomau( Ewing, I). Connor (4) and Kolodny (2) mention that in a small percentage of cases some myeloid and plasma cells are to be found in the tumor tissue, though neither author describes the method of identification of these cells, nor their relation to the neoplastic cells. In the case to be reported, there was a large primary tumor of the ilium, with skeletal metastases almost completely replacing the bone marrow, and little visceral involvement. These are unusual features for a Ewing sarcoma, but other evidence leads to that diagnosis. Upon the application of special staining methods, there was found scattered among the tumor cells an admixture of differentiated cells, apparently members of the hemopoietic series; and a proportion of the "undifferentiated" tumor cells seemed to resemble the myeloid stem-cell, the hemocytoblast. The possible significance of these findings will be discussed later in this paper. CASE REPORT R. C., a white male, aged nineteen years, entered the University of Chicago Clinics Aug. 9, 1928, complaining of (1) pain in the lower ex- tremities, especially the left, (2) a swelling in the region of the left ilium, 1 This work was done under a grant from the Douglas Smith Foundation for Medical Research. 386 UNDIFFERENTIATED ROUND CELL SARCOMA 387 (3) loss of vision in the right eye, and (4) gradual loss of weight and strength. Two years before admission he began to have transient sharp pains in the left hip region and down the back and outer side of the left leg, which were worse at night and gradually increased in severity. For the past ten months he had had a dull ache in his back, and fleeting pains in the right leg, back, shoulders, and face. Four months before admission he had suddenly lost most of the vision in the right eye, following a severe headache; and a little later he had developed a partial anesthesia of the right side of the face, which slowly disappeared. During the past six months he had gradually become pale, and had lost weight and strength. Tonsillectomy was done six months before admission because of x-ray findings of some disease of the left ilium. Three months before admission a mass was first palpated in that region; two exploratory operations were performed, leading to the diagnosis of osteomyelitis, though the wounds healed per primam, and no relief was obtained. The left leg had been weak for several months, and because of pain and general weakness the pat,ient had been confined to bed since the operations. Physical examination on admission disclosed a pale, weak, emaciated young man, suffering considerable pain. The right eye protruded slightly, and was nearly blind; the left eye showed a contraction of the visual field. The left pupil was the larger; both pupils reacted to light. There was a marked primary optic atrophy of the right eye; the other cranial nerves were objectively normal. No nodules were palpable on the skull, and the cervical lymph nodes were not enlarged. The lung areas were normal, as was the heart except for a soft, non-transmitted, systolic murmur at the apex. The abdomen was normal, except for a fulness of the left lower quadrant, where there was a firm, non-tender mass about the left ilium, filling out the iliac fossa, and laterally resulting in a moderate swelling of the gluteal region. On rectal examination the lower portion of this mass was palpable, filling out the left side of the pelvis; the prostate and seminal vesicles were normal. There were healed scars of operative incisions along the crest of the ilium, and just above the inguinal ligament. The left inguinal lymph nodes were moderately enlarged, firm, and slightly tender; nowhere else in the body were en- larged nodes detected. The muscles of the left leg were paretic, and mo- tion of the hip joint was painful and limited, but there was no sensory disturbance. Palpation of the entire skeleton revealed no other enlarge- ments nor areas of tenderness. The reflexes were normal except for a diminution of the left abdominal and left cremasteric responses. The white blood count was 19,000; the red blood count was 3,000,000, and the hemoglobin 60 per cent (Tallquist). The urine was repeatedly normal; no Bence-Jones protein was discovered. Blood Wassermann and Kahn tests were negative. Roentgenograms disclosed an increased thickness and density of the greater part of the left ilium and adjacent parts of the ischium and pubis. There was evidence of new bone formation on both surfaces of the ilium, appearing laminated on the outer surface, and more irregular 388 N. W. ROOME AND P. A. DELANEY and fuzzy on the inner surface (Fig. 1). No other lesions could be found at the first x-ray examination of the remainder of the pelvis, lumbar spine and sacrum, both femurs, chest, and skull. A tentative diagnosis of Ewing tumor of the ilium, with metastases in the region of the optic chiasm, was made. A series of 31 injections of Coley's toxin was given from Aug. 13 to Oct. 20, 1928, in doses increasing from 0.01 C.C.to 1.2 c.c., with febrile reactions after each, of 103" to 104.4' F. X-ray therapy was begun Aug. 30, 1928, a total of 600 Roentgen units being given, half anteriorly and half posteriorly, centered on the left ilium; a shorter course was also given over the right and left temporal regions, centered on the optic chiasm. Following this therapy the patient felt better. He had less pain and more function in the legs, and the mass in the pelvis decreased slightly in size. The x-ray findings remained almost unchanged. The eyes gradually UNDIFFERENTIATED ROUND CELL SARCOMA 389 became more prominent, and in October the patient complained of numb- ness of the left buccal mucous membrane. On Nov. 4, 1928, an oval tumor about 1.5 X 2.0 cm. was discovered on the right clavicle approximately 3 cm. from its sternal end; a roentgeno- gram showed at the corresponding point evidence of slight periosteal new bone formation with irregular reduction of the bone density beneath. A biopsy was done and a soft grayish tumor was found, which had eroded the bone and projected beyond its surface. Microscopic examination showed it to be made up of masses of closely packed round cells, with oval vesicular nuclei containing scattered finely granular chromatin, and non-staining cytoplasm; there were occasional mitotic figures (Fig. 2). The diagnosis of undifferentiated round-cell sarcoma (Ewing tumor) was thus confirmed. FIG. 2. TISSUEOBTAINED BY BIOPSYOF THE RIGHTCLAVICLE, NOV. 1928. X 835 X-ray studies in November and December showed small scattered areas of reduced density in cortex and medulla of both humeri, femora, tibiae and fibulae, and some periosteal new bone formation in the upper thirds of the humeri; there were no changes in the bones of the forearm and hand. There was irregular reduction of density of the sacrum and right and left pubic and ischial bones. The appearance of the left ilium was little altered. The humeri, right clavicle, and right pelvis were given short courses of x-ray therapy. The red blood count, Nov. 7, was 2,800,000. After December the condition became progressively worse; t,he mass in the pelvis enlarged and there were at times increasingly severe pains throughout the body. In January 1929 the patient suffered minor accidental burns of the abdomen, chest, and hands, which healed slowly. A differential white blood count on Jan. 25 showed 74 per cent polymor- phonuclear leukocytes, 3 per cent large lymphocytes, and 23 per cent small lymphocytes; no abnormal cells were described in the smears. 390 N. W. ROOME AND P. A. DELANEY Edema of the feet and ankles appeared in February and progressed to an extreme degree on the left side; in March the abdomen became distended, and free fluid and rhles were found in the chest. In April decline was even more rapid; on April 9 the white blood count was 8,600, the red blood count 1,260,000, and the hemoglobin 30 per cent. Palpable metastatic nodules appeared on the skull. The patient became incontinent and comatose, and died April 22, 1929. Throughout the illness the mouth temperature had fluctuated irregularly between 98" and 101" F. Necropsy Findings: Necropsy was done about two hol~rsafter death, by Dr. H. G. Wells. The body was extremely emaciated; there was little subcutaneous or omental fat. The mucous membranes, skin, muscles, and viscera were very pale; the skeletal muscles were atrophic. There was moderate edema of the lower extremities. A small amount of clear watery fluid was found in the abdominal cavity and there were a few fibrinous adhesions.
Load more

Recommended publications
  • Advances in Immune Checkpoint Inhibitors for Bone Sarcoma Therapy T Pichaya Thanindratarna,B, Dylan C
    Journal of Bone Oncology 15 (2019) 100221 Contents lists available at ScienceDirect Journal of Bone Oncology journal homepage: www.elsevier.com/locate/jbo Review Article Advances in immune checkpoint inhibitors for bone sarcoma therapy T Pichaya Thanindratarna,b, Dylan C. Deana, Scott D. Nelsonc, Francis J. Horniceka, ⁎ Zhenfeng Duana, a Department of Orthopedic Surgery, Sarcoma Biology Laboratory, David Geffen School of Medicine, University of California, 615 Charles E. Young. Dr. South, Los Angeles, CA 90095, USA b Department of Orthopedic Surgery, Chulabhorn hospital, HRH Princess Chulabhorn College of Medical Science, Bangkok, Thailand c Department of Pathology, University of California, Los Angeles, CA, USA ARTICLE INFO ABSTRACT Keywords: Bone sarcomas are a collection of sporadic malignancies of mesenchymal origin. The most common subtypes Immune checkpoint include osteosarcoma, Ewing sarcoma, chondrosarcoma, and chordoma. Despite the use of aggressive treatment Immunotherapy protocols consisting of extensive surgical resection, chemotherapy, and radiotherapy, outcomes have not sig- Bone sarcoma nificantly improved over the past few decades for osteosarcoma or Ewing sarcoma patients. In addition, chon- Anti-PD-1/PD-L1 drosarcoma and chordoma are resistant to both chemotherapy and radiation therapy. There is, therefore, an Anti-CTLA-4 urgent need to elucidate which novel new therapies may affect bone sarcomas. Emerging checkpoint inhibitors have generated considerable attention for their clinical success in a variety of human cancers, which has led to works assessing their potential in bone sarcoma management. Here, we review the recent advances of anti-PD-1/ PD-L1 and anti-CTLA-4 blockade as well as other promising new immune checkpoint targets for their use in bone sarcoma therapy.
    [Show full text]
  • Malignant Bone Tumors (Other Than Ewing’S): Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up by Spanish Group for Research on Sarcomas (GEIS)
    Cancer Chemother Pharmacol DOI 10.1007/s00280-017-3436-0 ORIGINAL ARTICLE Malignant bone tumors (other than Ewing’s): clinical practice guidelines for diagnosis, treatment and follow-up by Spanish Group for Research on Sarcomas (GEIS) Andrés Redondo1 · Silvia Bagué2 · Daniel Bernabeu1 · Eduardo Ortiz-Cruz1 · Claudia Valverde3 · Rosa Alvarez4 · Javier Martinez-Trufero5 · Jose A. Lopez-Martin6 · Raquel Correa7 · Josefina Cruz8 · Antonio Lopez-Pousa9 · Aurelio Santos10 · Xavier García del Muro11 · Javier Martin-Broto10 Received: 7 July 2017 / Accepted: 15 September 2017 © The Author(s) 2017. This article is an open access publication Abstract Primary malignant bone tumors are uncommon of a localized bone tumor, with various techniques available and heterogeneous malignancies. This document is a guide- depending on the histologic type, grade and location of the line developed by the Spanish Group for Research on Sar- tumor. Chemotherapy plays an important role in some che- coma with the participation of different specialists involved mosensitive subtypes (such as high-grade osteosarcoma). in the diagnosis and treatment of bone sarcomas. The aim is In other subtypes, historically considered chemoresistant to provide practical recommendations with the intention of (such as chordoma or giant cell tumor of bone), new targeted helping in the clinical decision-making process. The diag- therapies have emerged recently, with a very significant effi- nosis and treatment of bone tumors requires a multidiscipli- cacy in the case of denosumab. Radiation therapy is usually nary approach, involving as a minimum pathologists, radi- necessary in the treatment of chordoma and sometimes of ologists, surgeons, and radiation and medical oncologists. other bone tumors. Early referral to a specialist center could improve patients’ survival.
    [Show full text]
  • Primary Bone Cancer a Guide for People Affected by Cancer
    Cancer information fact sheet Understanding Primary Bone Cancer A guide for people affected by cancer This fact sheet has been prepared What is bone cancer? to help you understand more about Bone cancer can develop as either a primary or primary bone cancer, also known as secondary cancer. The two types are different and bone sarcoma. In this fact sheet we this fact sheet is only about primary bone cancer. use the term bone cancer, and include general information about how bone • Primary bone cancer – means that the cancer cancer is diagnosed and treated. starts in a bone. It may develop on the surface, in the outer layer or from the centre of the bone. As a tumour grows, cancer cells multiply and destroy The bones the bone. If left untreated, primary bone cancer A typical healthy person has over 200 bones, which: can spread to other parts of the body. • support and protect internal organs • are attached to muscles to allow movement • Secondary (metastatic) bone cancer – means • contain bone marrow, which produces that the cancer started in another part of the body and stores new blood cells (e.g. breast or lung) and has spread to the bones. • store proteins, minerals and nutrients, such See our fact sheet on secondary bone cancer. as calcium. Bones are made up of different parts, including How common is bone cancer? a hard outer layer (known as cortical or compact Bone cancer is rare. About 250 Australians are bone) and a spongy inner core (known as trabecular diagnosed with primary bone cancer each year.1 or cancellous bone).
    [Show full text]
  • Immunological Status of Peripheral Blood Is Associated with Prognosis in Patients with Bone and Soft-Tissue Sarcoma
    ONCOLOGY LETTERS 21: 212, 2021 Immunological status of peripheral blood is associated with prognosis in patients with bone and soft-tissue sarcoma YOUNGJI KIM1‑3, EISUKE KOBAYASHI1, YOSHIYUKI SUEHARA2, AYUMU ITO4, DAISUKE KUBOTA1,2, YOSHIKAZU TANZAWA1, MAKOTO ENDO1, FUMIHIKO NAKATANI1, TETSUYA NAKATSURA3, AKIRA KAWAI1, KAZUO KANEKO2 and SHIGEHISA KITANO3,5,6 1Division of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo 104‑0045; 2Department of Orthopedic Surgery, Juntendo University School of Medicine, Tokyo 113‑8431; 3Division of Cancer Immunotherapy, Exploratory Oncology Research and Clinical Trial Center, National Cancer Center Hospital, Tokyo 104‑0045; Departments of 4Hematopoietic Stem Cell Transplantation, and 5Experimental Therapeutics, National Cancer Center Hospital, Tokyo 104‑0045; 6Division of Cancer Immunotherapy Development, Advanced Medical Development Center, The Cancer Institute Hospital of Japanese Foundation for Cancer Research, Tokyo 135‑8550, Japan Received June 27, 2020; Accepted October 23, 2020 DOI: 10.3892/ol.2021.12473 Abstract. Immune‑checkpoint inhibitors have shown Multivariate Cox regression analysis demonstrated that the promising antitumor effects against certain types of cancer. number of Tim‑3+ CD8+ T cells was associated with lower However, specific immune‑checkpoint inhibitors for patients DFS time. A significant association was also found between with sarcoma have yet to be identified, whereas the immu‑ the number of M‑MDSCs and progression‑free survival (PFS) nological status of peripheral blood in patients with bone time in patients with metastasis. The results suggested the sarcoma and soft‑tissue sarcoma (STS) remains unknown. occurrence of immune surveillance, which indicated that the In addition, it is unclear whether the immunological status host immune reaction against cancer existed in patients with from the peripheral blood could be used as a prognostic bone sarcoma and STS.
    [Show full text]
  • Conventional Chondrosarcoma James C
    Conventional Chondrosarcoma James C. Wittig, MD SSSarcoma Surgeon Orthopedic Oncologist General Information Ma lignant mesenc hyma l tumor o f cart ilag inous different iat ion. Conventional Chondrosarcoma is the most common type of chondrosarcoma (malignant cartilage tumor) Neoplastic cells form hyaline type cartilage or chondroid type tissue (Chondroid Matrix) but not osteoid If lesion arises de novo, it is a primary chondrosarcoma If superimposed on a preexisting benign neoplasm, it is considered a secondary chondrosarcoma Central chondrosarcomas arise from an intramedullary location. They may grow, destroy the cortex and form a soft tissue component. Peripheral chondrosarcomas extend outward from the cortex of the bone and can invade the medullary cavity. Peripheral chondrosarcomas most commonly arise from preexisting osteochondromas. Juxtacortical chondrosarcomas arise from the inner layer of the periosteum on the surface of the bone. It is technically considered a peripheral chondrosarcoma. Chondrosarcoma Heterogeneous group of tumors with varying biological behavior depending on grade, size and location Cartilage tumors can have similar histology and behave differently depending on location. For instance a histologically benign appearing cartilage tumor in the pelvis will behave aggressively as a low grade chondrosarcoma. Likewise, a histologically more aggressive hypercellular cartilag e tumor localized in a p halanx of a dig it may behave in an indolent, non aggressive or benign manner. There are low (grade I), intermediate (grade II) and high grade (grade III) types of conventional chondrosarcoma. Low grade lesions are slow growing and rarely metastasize . Low grade chondrosarcomas can be difficult to differentiate from benign tumors histologically. Clinical features and radiographic studies are important to help differentiate.
    [Show full text]
  • Bone & Soft Tissue
    14A ANNUAL MEETING ABSTRACTS and testicular atrophy with aspermatogenia (negative OCT3/OCT4 stain). There was 47 Comparison of Autopsy Findings of 2009 Pandemic Influenza evidence of acute multifocal bronchopneumonia and congestive heart failure. He carried A (H1N1) with Seasonal Influenza in Four Pediatric Patients two heterozygous mutations in ALMS1: 11316_11319delAGAG; R3772fs in exon 16 B Xu, JJ Woytash, D Vertes. State University of New York at Buffalo, Buffalo, NY; Erie and 8164C>T ter; R2722X in exon 10. County Medical Examiner’s Office, Buffalo, NY. Conclusions: This report describes previously undefined cardiac abnormalities in this Background: The swine-origin influenza A (H1N1) virus that emerged in humans rare multisystem disorder. Myofibrillar disarray is probably directly linked to ALMS1 in early 2009 has reached pandemic proportions and cause over 120 pediatric deaths mutation, while fibrosis in multiple organs may be a secondary phenomenon to gene nationwide. Studies in animal models have shown that the 2009 H1N1 influenza virus alteration. Whether and how intracellular trafficking or related signals lead to cardiac is more pathogenic than seasonal A virus, with more extensive virus replication and dysfunction is a subject for further research. shedding occurring the respiratory tract. Design: We report four cases of influenza A-associated deaths (two pandemic and two 45 Sudden Cardiac Death in Young Adults: An Audit of Coronial seasonal) in persons less than fifteen years of age who had no underlying health issues. Autopsy Findings Autopsy finding on isolation of virus from various tissue specimen, cocurrent bacterial A Treacy, A Roy, R Margey, JC O’Keane, J Galvin, A Fabre.
    [Show full text]
  • Spectrum of Bone Tumors in Chiang Mai University Hospital, Thailand According to WHO Classification 2002: a Study of 1,001 Cases
    Spectrum of Bone Tumors in Chiang Mai University Hospital, Thailand According to WHO Classification 2002: A Study of 1,001 Cases Jongkolnee Settakorn MD*, Suree Lekawanvijit MD*, Olarn Arpornchayanon MD**, Samreung Rangdaeng MD*, Pramote Vanitanakom MD*, Sarawut Kongkarnka MD*, Ruangrong Cheepsattayakorn MD*, Charin Ya-In MD*, Paul S Thorner MD*** * Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai ** Department of Orthopedic Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai *** Department of Laboratory Medicine, Hospital for Sick Children and University of Toronto, Toronto, Canada Objective: The aim of the present study was to determine the spectrum, frequency and demographics of bone tumors. Material and Method: A retrospective study of the 1,001 bone tumor specimens from the files at the Pathology Department of the Chiang Mai University Hospital, Thailand from 2000 to 2004. Results: From the study, 41 were non-neoplastic mass lesions, and 960 were neoplastic, with 856 (89%) as primary and 104 (11%) as metastatic tumors. In the primary tumor group, 654 (76%) cases were of hemato- logic origin, and 202 (24%) were non-hematologic. The most common benign bone tumors were giant cell tumor (n = 37), osteochondroma (n = 25), and chondroma (n = 15). The most common malignant bone tumors were lymphoma-leukemia (n = 583), metastatic malignancy (n = 104), plasma cell myeloma (n = 71), and osteosarcoma (n = 58). Conclusion: The present study showed a higher frequency of osteosarcoma (68%), lower frequencies of chondrosarcoma (12%) and Ewing sarcoma (4%) among primary non-hematologic malignant bone tumors when compared with similar studies based on Western patients. Whether these differences reflect differences in the ethnic population or in practice patterns remains to be determined.
    [Show full text]
  • 507 High Dimensional Flow Cytometry Analysis in Newly Diagnosed Acute
    J Immunother Cancer: first published as 10.1136/jitc-2020-SITC2020.0507 on 9 November 2020. Downloaded from Abstracts LAG-3, and CSF1R at the PM interface were associated with made R code, we performed dimensionality reduction, cluster- worse progression-free survival (PFS), while gene sets associ- ing, and pseudotime analysis. ated with productive T cell immune response were associated Results The IR-score discriminated NR and CR (p = 3e-02, with improved PFS (figure 4). AUC 0.84) after treatment with CD57 and KLRG1 accounting Conclusions In contrast to primary bone osteosarcoma for most of this difference (p = 2e-02, AUC = 0.79). Next ‘immune deserts,’ osteosarcoma PMs represent an ‘immune- we investigated CD8+ T cell populations that best correlated excluded’ TME where immune cells are present but are halted with response to chemotherapy. FlowSOM revealed seven at the PM interface. TILs can produce effector cytokines, sug- major clusters: naive and naive-like, CD28+KLRG1+ acti- gesting their capability of activation and recognition of tumor vated-effector, CD28+KLRG1+PD1+ dysfunctional, PD1 antigens. Our findings suggest cooperative immunosuppressive +CD57+ senescent effector-memory and two clusters of ter- mechanisms in osteosarcoma PMs that prevent TILs from pen- minally differentiated CD45RA+KLRG1+ cells. Since the acti- etrating into the PM interior, including immune checkpoint vation and differentiation states accounted for most of the molecule expression and the presence of immunosuppressive subpopulation variability, we grouped the clusters into resting myeloid cells. We identify cellular and molecular signatures (naive, naive-like), activated (activated-effector, dysfunctional), that are associated with PFS of patients, which could be and terminally differentiated cells (senescent effector-memory, potentially manipulated for successful immunotherapy.
    [Show full text]
  • A Rare Case of Adamantinoma in an 11-Year-Old Pouyan Kheirkhah1, Bartlomiej Radzik1*, Kyle Sonnabend2, Grace Guzman1 and Steven Garzon1
    ISSN: 2378-3656 Kheirkhah et al. Clin Med Rev Case Rep 2021, 8:351 DOI: 10.23937/2378-3656/1410351 Volume 8 | Issue 6 Clinical Medical Reviews Open Access and Case Reports CASE REPORT A Rare Case of Adamantinoma in an 11-Year-Old Pouyan Kheirkhah1, Bartlomiej Radzik1*, Kyle Sonnabend2, Grace Guzman1 and Steven Garzon1 1Department of Pathology, University of Illinois Hospital and Health System, Chicago, USA 2Department of Radiology, University of Illinois Hospital and Health System, Chicago, USA Bartlomiej Radzik, Department of Pathology (MC 847), University of Illinois Hospital Check for *Corresponding author: updates and Health System, University of Illinois at Chicago, 840 S. Wood St., Suite 130 CSN, Chicago, IL 60612, USA, Tel: (312)-996-3886 Abstract [3]. Osteofibrous dysplasia is a benign bone lesion that occurs almost exclusively in pediatric long bone. Adamantinoma is a low-grade malignant lesion most frequently arising in the tibial cortex. Approximately 27% Histologically it is characterized by irregular bony of adamantinomas present with multifocal lesions in the trabeculae set within bland fibrous stroma organized same bone and 10% of cases involve the ipsilateral fibula. in a storiform pattern and, by definition, lacks an We describe an 11-year-old male with no significant past epithelial component. Adamantinoma is classified medical history who presented to the clinic with a 2-year history of abnormal gait. Two years after the onset of into two forms that correlate to prognosis of the symptoms, a painful mass was noticed on his left shin. tumor. OFD-A is characterized by scattered epithelial Patient biopsy showed a nest of epithelial-like cells elements that and with an average age of incidence arranged in a palisading pattern that stained positive for of 14 years of age.
    [Show full text]
  • Sarcoma Fact Sheet
    Sarcoma Fact sheet What is sarcoma? A sarcoma is a rare type of cancer that begins in the connective tissues of the body such as fat, muscle, blood vessels, nerves, bone and cartilage. Sarcoma occurs when abnormal cells in these types of tissue grow in an uncontrolled way. Sarcomas can develop anywhere in the body. Although sarcomas are rare across all age groups, they are among the more common types of solid tumour in childhood. Types of sarcoma Soft tissue sarcoma Soft tissue sarcomas develop in soft tissues such as fat, muscle, blood vessels, lymphatic vessels, nerves, tendons and cartilage. There are more than 50 types of soft tissue sarcomas. In adults, the most common types are: Undifferentiated pleomorphic sarcoma (UPS): an aggressive type of soft tissue sarcoma with high incidence of local recurrence and metastasis, most common in the age group of 50-70 years. Leiomyosarcoma: a malignant smooth muscle tumour that arises most commonly in the limbs, abdomen and uterus. Liposarcoma: a malignant tumour that arises from fat cells, most commonly in the trunk, limbs and abdomen. Angiosarcoma: a malignant tumour that arises from blood vessels or lymphatic vessels. Prior radiotherapy is a risk factor for angiosarcoma, often with a median latency period of 10 years. Malignant peripheral nerve sheath tumour: a malignant tumour that arises in the lining of nerves, often in the deep tissue of the arms, legs and trunk.. Fibroblastic sarcoma (fibrosarcoma): a malignant tumour that develops in the fibrous tissues of the body, most commonly in the limbs, skin and trunk. Gastrointestinal stromal tumour: a common type of soft tissue sarcoma that starts in the digestive tract.
    [Show full text]
  • Imaging of Bone Sarcomas
    JN054_Jrnl_50408Cummi.qxd 4/12/07 12:23 AM Page 438 438 Original Article Imaging of Bone Sarcomas Judd E. Cummings, MD; J. Andrew Ellzey, MD; and Robert K. Heck, MD, Memphis, Tennessee Key Words Diagnostic Evaluation Bone sarcoma, imaging, staging Patient evaluation begins with a thorough history and physical examination. Most commonly, patients present Abstract with pain at the affected site. The pain usually does not Identification, staging, and treatment of bone sarcomas rely on both improve and may progress to become independent of clinical and imaging evaluations. Although conventional radiogra- phy remains the primary imaging modality for characterizing bone activity. Ultimately, the patient may experience pain at tumors, bone scintigraphy, computed tomography, magnetic rest or at night. resonance imaging, and positron emission tomography can each Although some tumors show a sex predilection (fe- add information for staging and treatment planning. (JNCCN male predominance with giant cell tumor and parosteal 2007;5:438–447) osteosarcoma), this rarely is useful for diagnosis. Similarly, race is of little diagnostic benefit except with Ewing’s sar- Although much less prevalent than other forms of can- coma, which is extremely rare in people of African de- cer, bone sarcomas pose a dilemma in both diagnosis and scent. In contrast, the patient’s age is very helpful in treatment. Fortunately, over the past several decades, the directing the differential diagnosis of a suspected bone advancement and refinement of modalities aimed at treat- tumor.14,15 Examples include primary osteosarcoma, which ing bone sarcomas, such as adjuvant chemotherapy and usually occurs between the ages of 10 and 25 years; Paget’s limb-sparing surgical techniques, have resulted in better osteosarcoma, between 55 and 80 years; Ewing’s sarcoma, 1–13 outcomes for many patients.
    [Show full text]
  • Treating Bone Cancer How Is Bone Cancer Treated?
    cancer.org | 1.800.227.2345 Treating Bone Cancer How is bone cancer treated? The main ways to treat bone cancer are: ● Surgery for Bone Cancer ● Radiation Therapy for Bone Cancer ● Chemotherapy for Bone Cancer ● Targeted Therapy and Other Drugs for Bone Cancer Common treatment approaches Often, more than one type of treatment is used for bone cancer. Your treatment plan will depend on the type of bone cancer, which bone it started in, its stage (extent), and other factors. ● Treating Specific Types of Bone Cancer Who treats bone cancer? Primary bone cancers are not common. Because of this, not a lot of doctors have extensive experience with them. Treating these cancers can be complex, so they are often best treated by a team of doctors (and often at major medical centers). Doctors on the treatment team might include: ● An orthopedic surgeon: a doctor who uses surgery to treat bone and joint problems. Often this is an orthopedic oncologist, an orthopedic surgeon who specializes in treating cancer of the bones and joints. ● A radiation oncologist: a doctor who uses radiation to treat cancer 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 ● A medical oncologist: a doctor who uses chemotherapy and other medicines to treat cancer ● A physiatrist: a doctor specializing in rehabilitation and physical therapy Many other medical specialists may be involved in your care as well, including physician assistants (PAs) nurse practitioners (NPs), nurses, psychologists, social workers, rehabilitation specialists, and other health professionals. ● Health Professionals Associated with Cancer Care Making treatment decisions It's important to discuss all of your treatment options, including treatment goals and possible side effects, with your doctors to help make the decision that best fits your needs.
    [Show full text]