Relapsed primary extraskeletal osteosarcoma of liver:A case report and review of literature

BACKGROUND Extraskeletal osteosarcoma(ESOS)is a highly malignant osteosarcoma that occurs in extraskeletal tissues.It often affects the soft tissues of the limbs.ESOS is classified as primary or secondary.Here,we report a case of primary hepatic osteosarcoma in a 76-year-old male patient,which is very rare.CASE SUMMARY Here,we report a case of primary hepatic osteosarcoma in a 76-year-old male patient.The patient had a giant cystic-solid mass in the right hepatic lobe that was evident on ultrasound and computed tomography.Postoperative pathology and immunohistochemistry of the mass,which was surgically removed,suggested fibroblastic osteosarcoma.Hepatic osteosarcoma reoccurred 48 d after surgery,resulting in significant compression and narrowing of the hepatic segment of the inferior vena cava.Consequently,the patient underwent stent implantation in the inferior vena cava and transcatheter arterial chemoembolization.Unfortunately,the patient died of multiple organ failure postoperatively.CONCLUSION ESOS is a rare mesenchymal tumor with a short course and a high likelihood of metastasis and recurrence.The combination of surgical resection and chemotherapy may be the best treatment.

Extraskeletal Ewing sarcoma of the stomach:A rare case report

BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors.We herein present a rare case of ES involving the gastric area.CASE SUMMARY We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d.Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach;the mass was connected to the gastric antrum.Then,the mass was surgically excised completely.Upon histopathological,immunophenotype and molecular analysis,the mass was identified to be a primary gastric ES.CONCLUSION EES is an aggressive tumor with poor prognosis.Therefore,early diagnosis and timely intervention are essential for a good prognosis.It is imperative for us to raise awareness about this rare tumor.Surgical resection is still the best treatment option.

Extraskeletal myxoid chondrosarcoma metastatic to the pancreas: A case report

Extraskeletal myxoid chondrosarcoma (EMC) is a lowgrade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastatic sites are the lungs, soft tissues, lymph nodes, bones and the brain. To our knowledge, no case of clearly defined EMC has been reported to date developing a metastasis in the pancreas. We describe a case of a man suffering from EMC who developed a single pancreatic metastasis 20 years after the initial diagnosis. A 49-year-old man was submitted to surgical excision of an EMC, in left thigh, 20 years ago. Fourteen years after the initial diagnosis a local recurrence in left thigh occurred. Multiple lesions of metastatic origin, in both lungs, were excised via thoracotomies until the time being. In 2003, as a part of a periodically performed imaging control, an abdominal CT scan was performed revealing a solid lesion in the pancreas. Distal pancreatectomy was performed. The histopathology of the excised specimen proved to be the one of metastatic lesion of EMC. The above-mentioned case of EMC is, as far as we know, the first one described developing a certain pancreatic metastasis.

Clinicopathological Features of Extraskeletal Myxoid Chondrosarcoma:An Analysis of 9 Cases

Objective： To investigate the Clinicopathological （EMC）. Methods： Nine cases of extraskeletal characteristics of extraskeletal myxoid chondrosarcoma myxoid chondrosarcoma were studied. Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. Follow-up information was available for seven patients. Results： There were 7 males and 2 females whose ages ranged from 31 to 69 years （median 52.78 years）. Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients. The tumors were located mainly in the lower extremities （66.7%）. Most tumors were deep-seated. They usually had a distinct multinodular configuration delineated by fibrous connective tissue. The tumor cells were arranged in delicate intersecting strands, rings, and garlands for the most part. The myxoid matrix was abundant in most cases. Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. EMC expressed vimentin （100%, 9/9）, neuron-specific enolase （77.8%, 7/9）, S-100 protein （66.7%, 6/9）, synaptophysin and chromogranin A （22.2%, 2/9）. None of the tumors expressed EMA and desmin. Ultrastructurally： EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In many cells, there were also prominent glycogen deposits and lipid droplets. Some tumor cells had intracisternal microtubules. In one of the 2 extraskeletal myxoid chondrosarcomas there were 140-180 nm diameter membrane-bound dense-core secretory granules in cell bodies. Conclusion： Extraskeletal myxoid chondrosarcoma （EMC） is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features. However, the chondroid nature has been a subject of controversy, and its line of differentiation remains to be determined. A substantial proportion of EMC shows immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation. EMC has high potential of local recurrence and metastasis, and a high disease-associated death rate.

Perigastric extraskeletal Ewing's sarcoma: A case report

Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specifi c enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.

Extraskeletal Chondroma in the Popliteal Region: A Case Report

EXTRASKELETAL chondroma is a rare benigntumor that usually develops in the soft tissue,which commonly occurs in the limbs of adults andgenerally presents as painless masses without thesurrounding tissues involvement. The diagnosis mainlydepends on computed tomography, magnetic resonanceimaging （MRI） examination, and postoperative pathologicalexamination. This paper reports a case of extraskeletalchondroma in the popliteal region.

Extraskeletal Osteosarcoma of Penis: A Case Report

Extraskeletal osteosarcoma （EOS） is rare and commonly arises in the retroperitoneum, limbs, head and neck. There is no significant difference between EOS and other malignant tumors in soft tissue. Localized pain and swelling are the common presenting symptoms. Clinical diagnosis of EOS is difficult, imaging techniques may be helpful and careful, and the histopathological analysis is necessary. The common histological variants of EOS include： osteoblastoma, chondroblastoma, and fibroblastoma, and other unusual subtypes were reported occasionally. It should be distinguished with myositis ossificans, malignant mesenchymoma, giant cell tumor and parosteal osteosarcoma. We present an EOS arising in the penis. The primary site and histological category of the tumor were extremely rare. We hope the case will be helpful to the recognition of clinical signs, iconography and histopathology of EOS.

Primary intracranial extraskeletal myxoid chondrosarcoma:A case report and review of literature

BACKGROUND Primary intracranial extraskeletal myxoid chondrosarcoma(EMC)is an extremely rare low-to intermediate-grade malignant soft tissue sarcoma,and only 15 cases have been reported in the literature.Due to its rarity,clinical data and research on this tumor type are extremely limited,the pathogenesis and histological origin are still unclear,and the diagnostic and standard clinical treatment strategies for intracranial EMC remain controversial and undefined.CASE SUMMARY We reported a case of a 52-year-old male who was admitted to the hospital with headache and dizziness for 1 mo,and his health status deteriorated during the last week.CT of the head showed a well-defined low-density lesion situated in the left cavernous sinus.Brain magnetic resonance imaging(MRI)showed a 3.4 cm×3.0 cm sized,well-defined,round-shaped and heterogeneously enhanced lesion located in the left cavernous sinus.The entire lesion was removed via supratentorial craniotomy and microsurgery.Postoperative pathological diagnosis indicated primary intracranial EMC.Subsequently,the patient underwent 45 Gy/15 F stereotactic radiotherapy after discharge.At present,it is 12 mo after surgery,with regular postoperative follow-up and regular MRI examinations,that there are no clinical symptoms and radiographic evidence indicating the recurrence of the tumor,and the patient has returned to normal life.CONCLUSION Currently,the most beneficial treatment for primary intracranial EMC is gross total resection combined with postoperative radiotherapy.Long-term follow-up is also necessary for patients.

Gastrointestinal Stromal Tumor (GIST) with Chondroid-Myxoid-Chordoid Features Mimicking Extraskeletal Myxoid Chondrosarcoma

Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (PDGFRα) gene mutations, overexpress PDGFRα protein and respond to imatinib mesylate. Many cases of CD117-negative/CD117-weakly positive, c-kit mutation negative and PDGFRα mutation positive myxoid epithelioid GISTs and one case of CD117-positive GIST with chondro-myxoid features mimicking chondrosarcoma have been reported. We report a case of myxoid epithelioid GIST with predominance of chondroid and chordoid areas resembling an extraskeletal myxoid chondrosarcoma that was strongly positive for CD117, PDGFRα and DOG1 (Discovered on GIST 1) by immunohistochemistry, but lacked c-kit and PDGFRα gene mutations. It is possible that CD117 is strongly positive if a myxoid epithelioid GIST has chondroid/chordoid appearance, but a larger study is needed to confirm this association. CD117 expression in GISTs is important, because GISTs showing CD117 positivity respond to imatinib. No comment can be made about the prognostic significance of chondroid/chordoid appearance in the GISTs.

X-ray DIAGNOSIS OF EXTRASKELETAL (SOFT TISSUE) CHONDROSARCOMA (A REPORT OF 8 CASES)

Eight cases of surgically and pathologically verified extraskeletal (soft tissue) chondrosarcoma were analyzed with regard to clinical and X-ray features. The cardinal clinical aspects of this series are: presence of a local soft tissue mass; gradual enlargement of the mass accompanied by increasing pain. The X-ray signs were summarized as follows: formation of a soft tissue mass; various forms of calcifications concentrated in the central area of the tumor; in some instances, presence of a saucer-like defect on the cortical surface of neighbouring bone and periosteal proliferation with mound-like new bone on both sides as well as bending deformity of the affected bone. The incidence and sites of predilection, the main X-ray findings, radiological diagnosis and differential diagnosis of the tumor were discussed. The Roentgen features of synovial chondrosarcoma of the knee joint were especially analyzed.

Imaging-based diagnosis for extraskeletal Ewing sarcoma in pediatrics: A case report

BACKGROUND Extraskeletal Ewing sarcoma(EES)is a member of the Ewing sarcoma family of tumors which is pathologically known as a small,round,blue cell tumor involving bone and soft tissue.The prevalence of EES is only 15%-25%of all Ewing sarcoma and EES often occurs in patients aged from 20-mo-old to 30-yearsold resulting in an unfavorable prognosis.CASE SUMMARY The present case report described a 7-year-old patient with a palpable EES mass of 33 mm×27 mm×28 mm in the deep neck with symptoms of persistent dyspnea over the past 5 mo.After laboratory examinations,abnormal physiological and biochemical indicators were not found.Ultrasound images presented the mass to be complex,solid and fluid-filled with circumscribed margins and posterior acoustic enhancement.The mass also presented with partial internal vascularity.The contrast-enhanced magnetic resonance imaging scan illustrated the outstanding enhancement with fast perfusion mode in the early arterial phase. CONCLUSIONOur study suggested that a quick-growing mass in the pediatric patient is possibly a malignanttumor whether the mass has well-defined margins or not.

Primary extraskeletal Ewing’s sarcoma of the lumbar nerve root:A case report

BACKGROUND Ewing’s sarcoma(ES)is a highly aggressive bone malignancy.Extraskeletal ES(EES)originating in the spinal canal is extremely rare.Herein,we report on a rare case of EES with a primary lumbar spinal nerve root including the complete diagnosis and treatment.CASE SUMMARY A young female patient presented with a complaint of right lower limb pain for 1 mo.Magnetic resonance imaging(MRI)revealed an 11 mm×14 mm×31 mm mass in the lumbar epidural region extending at the fifth lumbar spine(L5)level toward the right L5 neural foramen.Our initial diagnosis was an epidural schwannoma.The patient underwent total laminectomy,tumor resection and pedicle screw internal fixation and the L5 root tumor was found to have been completely removed intraoperatively.Histopathological examination of the lesion showed a typical ES with a large number of small,round cells.Immunohistochemistry analysis indicated positive CD99 and S100.After surgery,the patient received chemotherapy and radiotherapy with a 1 year of follow-up and no recurrent tumors or new lesions were found upon spine MRI and positron emission tomography/computed tomography reexamination.CONCLUSION Clinically,ES outside the bone should be considered when nerve root tumors are encountered inside and outside the spinal canal and the diagnosis should be determined by pathological biopsy.After surgical resection,chemotherapy and radiotherapy should be performed.After treatment,active follow-up and regular review should be completed.

Retroperitoneal Extraskeletal Osteosarcoma in a Pregnant Woman Incidentally Found on Cesarean Section

Extraskeletal osteosarcoma (ESO) is a rare malignant mesenchymal neoplasm accounting for 1% - 2% of all soft tissue sarcomas that produce osteoid, bone, and/or chondroid material. Here we report the first case of a retroperitoneal ESO in a 35-year-old pregnant woman. A retroperitoneal ESO was incidentally found at cesarean section. Despite treatment, she rapidly died from wide spread dissemination. We focus on histological characteristics including immunohistochemical results and differential diagnosis of ESO.

Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage:A case report and review of the literature

Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor(E-EWS/pP NET) is a rare aggressive malignant small round cell tumor. In this report, we present the case of a 15-year-old boy who suffered from acute abdominal pain accompanied by hematemesis and melena, and was eventually diagnosed with E-EWS/p PNET. To date, there have been only five reported cases of E-EWS/pP NET of the small bowel including the patient in this report. To the best of our knowledge, this is the first documentation of a pP NET of the small bowel mesentery at nonage. All these have made this report rare and significant.

Giant abdominal osteosarcoma causing intestinal obstruction treated with resection and adjuvant chemotherapy

Extraskeletal osteosarcoma(ESOS)is an uncommon tumor that accounts for 1% of all soft tissue sarcomas and 4% of all osteosarcomas. Its presentation may be atypical,while pain has been described as the most common symptom. Radiological findings include a large mass in the soft-tissues with massive calcifications,but no attachment to the adjacent bone or periosteum. We present the case of a 73-year-old gentle man who presented with a palpable,tender abdominal mass and symptoms of bowel obstruction. Computer tomography images revealed a large space-occupying heterogeneous,hyper dense soft tissue mass involving the small intestine. Explorative laparotomy revealed a large mass in the upper mesenteric root of the small intestine,measuring 22 cm × 12 cm × 10 cm in close proximity with the cecum,which was the cause of the bowel obstruction. Pathology confirmed the diagnosis of an ESOS. ESOS is an uncommon malignant soft tissue tumor with poor prognosis and a 5-year survival rate of less than 37%. Regional recurrence and distant metastasis to lungs,regional lymph nodes and liver can occur within the first three years of diagnosis in a high rate(45% and 65% respectively). Wide surgical resection of the mass followed by adjuvant chemotherapy or radiotherapy has been the treatment of choice.

Primary chondrosarcoma of the liver:case report and literature review

We present a rare case of primary chondrosarcoma of the liver in a 57-year-old man with pre-existing hepatitis B virus-related chronic hepatitis.The MRI scans showed a huge cystic-solid occupation of 18 cm×17 cm×11 cm in the right hepatic lobe.The tumor was completely resected,and the histological findings identified low-grade cartilaginous component with typical ring-and-arc chondroid matrix mineralization.Immunohistochemically,the neoplastic cells were positive for vimentin and S-100 protein.The patient received once postoperative adjuvant transcatheter arterial chemoembolization(TACE)at the 2nd month after discharge,and he is still alive for more than 13 months without recurrence and metastasis.To the best of our knowledge,this is the first case of primary chondrosarcoma of the liver.

Primary chondrosarcoma of the liver:A case report

BACKGROUND Primary chondrosarcoma of the liver are extremely rare.Moreover,there are few reports focusing on typical clinical symptoms and imaging characteristics.Therefore,the diagnosis of chondrosarcoma of the liver remains a challenge.CASE SUMMARY A 59-year-old male was admitted due to a lesion occupying the right liver lobe that was found by physical examination.Magnetic resonance imaging showed a lobular mass with high T2 weighted image and low T1 weighted image with enhanced internal separation and edge in the right liver.He was diagnosed with liver cystadenoma by using magnetic resonance imaging.At 3 mo later,the magnetic resonance scan showed that the mass was enlarged.Laparoscopic liver tumor resection was performed with a pathological diagnosis of liver chondrosarcoma.Then he received a surgical resection for the recurrent lesion.However,intrahepatic and abdominal metastases were found again at 8 mo after the second operation.The patient then received conservative management and is now under follow-up.CONCLUSION Primary liver chondrosarcoma generally is presented as lobulated and heterogeneous density/signal,cystic,solid masses without calcification with enhanced edge,internal septa and solid part.The imaging features are closely related to pathology,which may be helpful for clinical diagnosis.

Primary Chondrosarcoma of the Mammary Gland of a Spayed American Akita Bitch: A Case Report

An eleven-year-old spayed American Akita bitch rapidly developed a mammary tumour which was surgically removed. The histological examination revealed the tumour to be a sarcoma that presented positive immunohistochemical staining against vimentin and protein S 100. There was no staining against pan-actin. Based on the histological aspect and the pattern of immunohistochemical staining the diagnosis of primary extraskeletal chondrosarcoma of the mammary gland was considered most probable. Two months later the patient was euthanized with recurrent local tumour and metastases in the lungs.

Two Cancers Arising from Different Mesenchymal Origins: An Unexpected Connection over Time and Space

Sarcomas are rare tumours, accounting for 1% of all malignancies. They are histologically diverse, presenting with more than 50 different subtypes. Sarcoma can be broadly divided into two categories—soft tissue sarcoma and bone sarcoma. Extraskeletal osteosarcoma is a unique subtype which is histologically similar to bone osteosarcoma but resides within soft tissue and has no attachments to bone. We present a rare case of a patient who initially presented with an extraskeletal osteosarcoma that was complicated by a chronic chest wall sinus and lymphedema of the left arm. He later developed enlarging left chest wall nodules at the same region that were proven to be dedifferentiated liposarcoma on biopsy 20 years later. We examine the occurrence of both extremely rare diseases of differing pathologies over the same site but separated by an extraordinarily long interval, and possible new associations to account for this phenomenon.