Cases reported "Sarcoma"

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1/20. Delay in the diagnosis and treatment of primary bone sarcoma of the pelvis.

    BACKGROUND: Symptoms arising from primary bone sarcoma of the pelvic girdle are often insidious in onset and nonspecific in nature. To make the subtle initial signs and symptoms of these tumors more apparent to clinicians, we studied a cohort of patients who had a primary bone sarcoma of the pelvic girdle. Our purpose was to describe the initial clinical findings and to evaluate the duration, frequency, and implications of delays in the treatment of these tumors. methods: We retrospectively analyzed the data on sixty-eight patients who had a primary bone sarcoma of the pelvic girdle. The data that we reviewed included demographic characteristics; histological diagnosis; anatomical location, size, and stage of the tumor; characteristics of the biopsy specimen; duration and description of symptoms before an accurate diagnosis was made; delay before recognition of the tumor on radiographs; results of diagnostic imaging; inaccurate diagnoses; type of intervention based on these inaccurate diagnoses; and outcome with regard to survival. There were forty high-grade sarcomas and twenty-eight low-grade sarcomas. RESULTS: Excluding two asymptomatic patients in whom the sarcoma was noted incidentally, the average duration of symptoms before an accurate diagnosis was made was ten months (median, six months; range, one month to four years). Common symptoms and findings on physical examination included pain in the buttock (twenty-three patients; 35 percent), a mass (twenty patients; 30 percent), sciatica (nineteen patients; 29 percent), pain in the hip (seventeen patients; 26 percent), pain in the groin (thirteen patients; 20 percent), and low-back pain (fourteen patients; 21 percent). In thirty (44 percent) of the sixty-eight patients, the pelvic sarcoma was not recognized initially and an inaccurate diagnosis was made. The misdiagnoses included a herniated lumbar disc, spinal stenosis, spondylolisthesis, tendinitis, bursitis, an inguinal hernia, a stress fracture, a pilonidal cyst, a recurrent urinary-tract infection, and degenerative arthritis of the spine, hip, and knee. Inappropriate treatment for these misdiagnoses included seven operative procedures (two laminectomies, two debridements, one hip arthrotomy, one total knee replacement, and one inguinal herniorrhaphy), six courses of nonsteroidal anti-inflammatory medications, five chiropractic adjustments, four trials of physical therapy, and three local injections of steroids. It took an average of seven months for the clinicians to arrive at the diagnosis of primary pelvic sarcoma. With the numbers available, no significant association between the duration of symptoms before an accurate diagnosis was made and the grade or the stage of the tumor could be detected. In addition, no association between the duration of symptoms and the survival of the patient (p = 0.54) could be determined, with univariate analysis. The grade and the stage of the tumor were strongly associated with the outcome, with a low tumor grade proving to be a favorable prognostic indicator for survival (p = 0.006). CONCLUSIONS: patients who have a primary bone sarcoma of the pelvis often have had symptoms for a long duration that mimic those of more commonly encountered non-neoplastic musculoskeletal conditions. When a patient has symptoms that appear to be out of the ordinary, particularly refractory pain or pain at rest, physicians should include the pelvic girdle in the evaluation and should carefully examine a high-quality radiograph of the entire pelvis.
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2/20. Case report of a patient with an intimal sarcoma of the pulmonary trunk presenting as a pulmonary embolism.

    A fatal case of an 89-year-old woman with an intimal sarcoma obstructing the pulmonary trunk and an open foramen ovale is presented. Clinical symptoms, physical examination and further evaluation originally raised suspicion of a pulmonary embolism. Recent classification systems, specific radiological and pathological characteristics of sarcomas of the pulmonary trunk are discussed.
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3/20. Cutaneous promyelocytic sarcoma at sites of vascular access and marrow aspiration. A characteristic localization of chloromas in acute promyelocytic leukemia?

    Extramedullary disease (EMD) is a rare clinical event in acute promyelocytic leukemia (APL). Although the skin is involved in half of the reported EMD cases, the occurrence of cutaneous promyelocytic sarcoma (PS) has been described very rarely. We report here three cases of PS which have the peculiarity of appearing at sites of punctures for arterial and venous blood and marrow samples (sternal manubrium, antecubital fossa, wrist over the radial artery pulse, catheter insertion scar). At presentation, all patients had hyperleukocytosis and a morphologic diagnosis of microgranular acute promyelocytic leukemia variant confirmed at the genetic level by demonstration of the specific chromosomal translocation t(15;17). A BCR3 type PML/RARa transcript was documented in the two patients for whom diagnostic RT-PCR was available. patients had morphologic bone marrow remission at the time the PS appeared. A predilection for the development of cutaneous PS at sites of previous vascular damage has been noted, but the pathogenesis remains largely unknown. A potential role for all-trans retinoic acid has been advocated, although one of the three patients in our series had received no ATRA. A review of the literature revealed six similar cases and hyperleukocytosis at diagnosis was a consistent finding in all of them. A careful physical examination of these particular sites in the follow-up of patients at risk, as well as cutaneous biopsy and laboratory examination of suspected lesions are strongly recommended.
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4/20. uterine artery embolization in an undiagnosed uterine sarcoma.

    BACKGROUND: Uterine sarcomas are rare malignancies that resemble benign uterine leiomyomata. uterine artery embolization is offered increasingly for treatment of uterine leiomyomata, which might lead to embolization of undiagnosed uterine sarcoma. CASE: A 52-year-old woman, gravida 7, para 6, with perimenopausal menometrorrhagia was diagnosed with uterine leiomyomata after physical examination and transvaginal ultrasound. An endometrial biopsy was negative for malignancy. After medical treatment was unsuccessful, she had uterine artery embolization. She then passed a piece of tissue from her vagina, the pathology report of which was necrotic high-grade sarcoma. During surgery we confirmed that the tumor was confined to the uterus. CONCLUSION: Uterine sarcoma cannot be diagnosed except by pathologic examination of a resected specimen. women considering uterine artery embolization for treatment of apparent leiomyomata should be counseled on the risk of decreased survival by delaying diagnosis and treatment of uterine sarcoma.
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5/20. Primary C1-2, intradural, extramedullary meningeal sarcoma with glial fibrillary acidic protein-immunoreactive components: a spinal gliosarcoma? Case report and review of the literature.

    The authors report an exceedingly rare case of a patient harboring a primary, spinal, C1-2, intradural, extramedullary meningeal sarcoma in which there were glial fibrillary acidic protein-immunoreactive components, but, importantly, no physical connection with neural tissue. On initial diagnostic imaging, neuroradiological features suggestive of a spinal meningioma were demonstrated in this 73-year-old man. He underwent a C1-2 laminectomy and removal of the posterior ring of the foramen magnum, and the lesion was excised. Histological and immunohistochemical testing showed a gliosarcoma. The clinical, radiological, and operative data are reviewed, as are the histopathological findings. To the authors' knowledge, this is the first case of a primary spinal intradural extramedullary gliosarcoma reported in the literature.
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6/20. Free fibula long bone reconstruction in orthopedic oncology: a surgical algorithm for reconstructive options.

    The fibula free flap became popular in orthopedic oncology for limb-sparing long bone tumor resection. It is particularly suitable for intercalary or resection arthrodesis options. In the present series, a surgical reconstruction algorithm was used, enabling each patient to receive a personalized technique. During the years 1998 to 2002, 30 patients underwent limb-sparing surgery for long bone sarcoma. There were 18 males and 12 females. Their mean age was 23 years (range, 9 to 70 years). The diagnoses were Ewing's sarcoma (11 patients), osteogenic sarcoma (eight patients), chondrosarcoma (five patients), giant cell tumor of bone (three patients), high-grade soft-tissue sarcoma (two patients), and leiomyosarcoma of bone (one patient). The majority of tumors where located in the lower extremity (23 patients), mostly in the femur (15 patients with four tumors in the proximal femoral shaft, five tumors in the distal femoral shaft, five tumors in the whole femoral shaft, and one tumor in the proximal femoral head). In seven patients, the upper extremity was involved; in six patients, the radius was involved; and in one patient, the humerus was involved. The free fibula flap was used in three types of approaches: vascularized fibula as an osseous flap only (18 patients), a combination of a vascularized fibula flap in conjunction with an allograft (Capanna's technique; 10 patients), and a free double-barreled fibula (two patients). All flaps survived. Postoperatively, all patients were monitored clinically, radiologically, and by radioisotope bone scan studies. Callus formation and union were shown 2.6 to 8 months postoperatively. patients who underwent lower extremity reconstruction were nonweightbearing for 3 to 9 months, with a transition period in which they used a brace and gradually increased weightbearing until full weightbearing was achieved. Eight patients had 11 recipient-site complications. Two patients (6.7 percent) had hematomas, and three patients (10 percent) had infection and dehiscence of the surgical wound with bone exposure in one patient; all complications resolved with conservative treatment only. Failure of the hardware fixation system occurred in two patients, mandating surgical correction. No fibula donor-site complications were recorded. In intercalary resections, the use of the vascularized fibula flap as an isolated osseous flap might be insufficient. Different body sites have different stress loads to carry, depending on the age of the patient and on his individual physical status. To achieve initial strength in the early period, the authors combined the free fibula flap with an allograft (Capanna's method) or augmented it as a double-barreled fibula. They propose a surgical algorithm to assist the surgeon with the preferred method for reconstruction of various long bone defects in different body locations at childhood or adulthood. Long bone reconstruction using a vascularized fibula flap, alone or in combination with an allograft, autogenous bone graft, or double-barreled fibula for limb-sparing surgery, is a safe and reliable method with a predictable bony union, good functional outcome, and a low complication rate.
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keywords = physical
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7/20. Primary breast sarcoma: case report.

    Primary breast sarcoma is a rare entity occurring in 0.5% of women with breast malignancy. Like in breast carcinoma, delay in its diagnosis has important clinical and treatment implications. The subject of this report presented at our breast unit with advanced breast lesion months after she noticed a small lump in her right breast. She had no clear diagnosis despite several consultations, in-patient treatments at two facilities in the city, breast ultrasonography, breast mammography and three fine needle aspiration cytology (FNAC) examinations. The patient needed multiple blood transfusions. A final FNAC showed ductal carcinoma. histology following wide excision confirmed high-grade primary stromal breast sarcoma. She required adjuvant combination chemotherapy. A combination of diagnostic failures and patient fault caused delay in subject's treatment. Lesion progression during delay which influenced the pattern of physical morbidity, tumour prognosis and need for adjuvant treatment. Embracing the concept of breast care in dedicated breast units may minimise such treatment delays.
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8/20. Undifferentiated (embryonal) sarcoma of liver in adult: a case report.

    We report a case of undifferentiated (embryonal) sarcoma of the liver (UESL), which showed cystic formation in a 20-year-old man with no prior history of any hepatitis or liver cirrhosis. He was admitted with abdominal pain and a palpable epigastric mass. The physical examination findings were unremarkable except for a tenderness mass and the results of routine laboratory studies were all within normal limits. Abdominal ultrasound and computed tomography (CT) both showed a cystic mass in the left hepatic lobe. Subsequently, the patient underwent a tumor excision and another two times of hepatectomy because of tumor recurrence. Immunohistochemical study results showed that the tumor cells were positive for vimentin, alpha-1-antichymotrypsin (AACT) and desmin staining, and negative for alpha-fetoprotein (AFP), and eosinophilic hyaline globules in the cytoplasm of some giant cells were strongly positive for periodic acid-Schiff (PAS) staining. The pathological diagnosis was UESL. The patient is still alive with no tumor recurrence for four months.
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keywords = physical examination, physical
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9/20. Gastrointestinal stromal tumor of the prostate.

    We present a unique case of an atypical prostate tumor in a 49-year-old man with acute urinary retention. digital rectal examination revealed a large prostate with a parenchymal mass on the left side. Radiologic imaging showed a 14.2 x 9.6 x 14.0-cm prostatic mass and multiple liver metastases. A pathologic diagnosis of a prostatic gastrointestinal stromal tumor was made and was confirmed by molecular analysis. Therapy consisted of a tyrosine kinase inhibitor, resulting in prostate mass reduction. After 100 weeks, the patient was in good physical condition with a continuing partial response with a reduced mass volume and liver nodules.
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10/20. Non-puerperal uterine inversion in association with uterine sarcoma: case report in a 26-year-old and review of the literature.

    INTRODUCTION: Inversion of the uterus associated with a uterine sarcoma is extremely rare with only 17 cases reported since 1887, so that its clinical presentation and appearance at laparotomy may be confusing. CASE: A 26-year-old woman presented with a vaginal mass and bleeding. At laparotomy, her uterus appeared depressed or concave. The final diagnosis of uterine sarcoma and inversion was unique in her age group. CONCLUSIONS: Uterine sarcoma with inversion can occur in the premenopausal age group. It presents as a vaginal mass with bleeding. As a clue to the diagnosis, the uterus per se may not be palpable or it may appear concave on physical exam or at laparotomy.
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