UNUSUAL GASTROINTESTINAL METASTASES FROM AN ALVEOLAR SOFT PART SARCOMA

Alveolar soft part sarcoma (ASPS) is a rare subtype of soft tissue sarcoma that occurs predominantly in young patients. Despite its relatively indolent course, it generally has a poor prognosis with widespread metastases. The common metastatic sites from an ASPS include the lung, brain and bone. However, metastasis of an ASPS to the gastrointestinal tract is extremely rare. Here, we report a rare case of upper gastrointestinal bleeding and jejunal intussusception due to gastrointestinal metastases from an ASPS.     

Alveolar soft part sarcoma in brain with cardiac metastasis: a case report

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The coexistence of ASPS with cardiac metastasis is quite rare, in particular. In general, the sarcoma is a malignant disease and grows very fast. However, the mean survival time of patients with ASPS is relatively long. Patients who are diagnosed with localized disease usually have a favorable prognosis, while those who present with widespread metastases usually have a poor prognosis and ultimately, succumb to their disease. The use of modern treatment modalities, such as combination of surgery, and radiotherapy, may significantly prolong the survival time in many patients. Because of the long-term period with symptomatic brain tumors in this disease, the patients should be treated even in the presence of multiple metastases in other organs.     

Malignant fibrous histiocytoma of peritoneum presenting as intestinal obstruction.

Malignant fibrous histiocytoma (MFH) is a high-grade soft-tissue sarcoma of fibroblast-cell origin with a propensity for metastasis and recurrence. Primary MFH of the peritoneum is rare. We report a 60-year-old man with MFH of the peritoneum presenting with obstructive symptoms. Complete surgical excision of the tumor was done, and he is well six months later.     

Risk Factors for Lymphatic Metastasis of Malignant Bone and Soft-Tissue Tumors: A Retrospective Cohort Study of 242 Patients

Metastasis to the lymph nodes is relatively rare in malignant bone and soft-tissue tumors, and its risk factors remains unknown, except for tumors of the lymphogenous histotype, including rhabdomyosarcoma, epithelioid sarcoma, and clear cell sarcoma. The purpose of this study was to identify the risk factors for lymph node metastasis of malignant bone and soft-tissue tumors.We retrospectively reviewed 242 patients with malignant bone and soft-tissue tumors. The predictors of interest for the risk of lymph node metastasis included age, sex, histopathological diagnosis, location(s) of the primary tumor(s), local recurrence, residual tumor(s), and the size of the primary tumors. To identify the risk factors for lymph node metastasis, Cox regression analyses were performed.Among the 242 patients with malignant bone and soft-tissue tumors in the current study, 60, 29, and 28 were detected to have lung, lymph node, and bone metastases, respectively. In the univariate analyses, the lymphogenous histotype and a primary tumor invading the subcutis were the risk factors for lymph node metastasis. In the multivariate analysis, the lymphogenous histotype (P < 0.01) and a primary tumor in the subcutis (P < 0.01) remained significantly associated with a higher risk of lymph node metastasis with 5.15 and 3.48 of hazard ratios, respectively.Lymph node metastasis was detected in malignant bone and soft-tissue tumors more frequently than that has been previously reported, and the risk factors for lymph node metastasis were the lymphogenous histotype and primary tumors invading the subcutis.     

Malignant fibrous histiocytoma of the gastrointestinal tract in a patient with neurofibromatosis

Malignant fibrous histiocytoma (MFH) of the gastrointestinal tract is extremely rare. A case of MFH of the colon associated with neurofibromatosis is presented. MFH is a high-grade soft-tissue sarcoma of fibroblast cell origin with a strong propensity for metastasis and recurrence. Immunochemical markers help to differentiate MFH from other sarcomas. The most successful treatment of MFH is surgical extirpation. Adjuvant chemotherapy and radiotherapy have not been definitively shown to be of value.     

腹膜后腺泡状软组织肉瘤1例

腺泡状软组织肉瘤是一种少见的软组织恶性肿瘤,发生于腹膜后的腺泡状软组织肉瘤极为罕见,主要表现为无痛性生长的软组织肿块.国内外报道罕见,且易与副神经节瘤、腺泡状横纹肌肉瘤、肾透明细胞癌、颗粒细胞瘤混淆.本文报道腹膜后的腺泡状软组织肉瘤1例,结合影像学、临床病理学特征、免疫组织化学及基因检测技术,作出了正确诊断及治疗,并结合相关文献进行分析讨论,进一步提高了对腹膜后的腺泡状软组织肉瘤的认识.     

Fibromyxoid sarcoma of the kidney. A case report

Low grade fibromyxoid sarcoma is an uncommon soft tissue tumor. We present the case of a 83-year-old female who developed large mass in the right kidney. A nephrectomy was performed. The tumor measured 18,5x17,5x11,5 cm and on pathology evaluation was diagnosed as low grade fibromyxoid sarcoma. Currently, the patient is doing well without evidence of local recurrence and distant metastasis. Low grade fibromyxoid sarcoma of the Kidney is a very rare tumor. The metastasizing potential is high. Sometimes long interval between tumor presentation and metastasis is observed. The treatment of choice is surgery. The role of radiotherapy and chemotherapy is uncertain.     

Presentation of gastric cancer as a soft tissue tumor

Soft tissue metastasis of gastric cancer is extremely rare. We report an 82 year old male, who was being evaluated for benign prostate hyperplasia and incidentally a soft tissue mass in the right buttock was discovered. Differential diagnosis included neurofibroma vs. soft tissue sarcoma. Patient underwent surgical resection and pathologic analysis reported adenocarcinoma with ring cell differentiation. Postoperative work-up included a CT scan of abdomen and pelvis and upper gastrointestinal endoscopy. An ulcer in the gastric antrum was discovered and biopsied. Pathology reported the same tumor as the soft tissue mass. PET scan demonstrated extensive metastatic disease. We reviewed the literature looking for other cases of soft tissue metastasis of gastric cancer.     

Significance of immunohistochemistry and FISH of TFE3 in the diagnosis of alveolar soft part sarcoma: A case report

Rationale:Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma harboring an ASPL-TFE3 fusion gene. Herein, we report a case of ASPS associated with brain metastasis. Immunohistochemistry (IHC) for TFE3 antigen expression and fluorescence in situ hybridization (FISH) for TFE3 rearrangement were performed to arrive at an accurate diagnosis.Patient concerns:A 47-year-old man was hospitalized for a headache and numbness of the lower limbs.Diagnoses:Preoperative computed tomography and magnetic resonance imaging revealed 2 brain masses, 1 each in the right parietal and temporal bones. We diagnosed this case as ASPS with brain metastasis based on histological morphology, IHC, and FISH.Interventions:The patient underwent right skull titanium mesh implantation and supratentorial superficial lesion resection.Outcomes: The patient recovered well after discharged from hospital.Lessons:The diagnosis of ASPS depends on careful clinical, radiographic, histopathological, IHC, and FISH assessments to arrive at the correct diagnosis. Thus, TFE3 may be useful in the diagnosis and treatment of ASPS.     

Synovial sarcoma of the hard palate: The third case in the medical literature

Synovial sarcoma is a high-grade soft tissue sarcoma that rarely arises in the head and neck region. It affects the parapharyngeal space and the hypopharynx most commonly and it has different presentations based on the affected site. In extremely rare occasions, it involves the hard palate such as in our case where a 24-year-old female patient presented with a mass lesion involving the left hard palate, which was identified clinically and by imaging studies. The histopathological assessment confirmed that it was a monophasic synovial sarcoma which was also confirmed with further molecular studies. The patient underwent surgical excision and postoperative radiotherapy. Her close follow up over a 6-year period that followed her curative treatment has demonstrated no evidence of disease recurrence or distant metastasis. Surgical excision is the mainstay of treatment for synovial sarcoma and adjuvant radiotherapy is advised. Long-term follow up is recommended because of the remote possibility of late recurrence of the tumor.     

A case report of multiple pulmonary tumors as a sole manifestation of synovial sarcoma]

A 47-year-old woman was admitted to our hospital for cough and dyspnea. Roentgenologic studies and bronchoscopy revealed multiple lung tumors one of which obstructed the right main bronchus. Right pneumonectomy was performed for the pending obstruction of the trachea. The tumor in the right S1 was found to be protruding into the trachea through the right B1 and the main bronchus in a polypoid fashion. The pathological diagnosis of synovial sarcoma was made on the basis of the characteristic biphasic structure composed of spindle cells and epithelioid cells forming gland-like spaces. Three years and eight months after the pneumonectomy, a nodule in the tendon of the extensor hallucis longus muscle became palpable. It was also a synovial sarcoma pathologically. Synovial sarcoma is a soft tissue sarcoma which usually arises in the extremities. It is very rare for pulmonary metastasis of this tumor to be found while the primary tumor is undetectable.     

Primary Cardiac Pleomorphic Sarcoma Presenting as Back Pain in an 18-Year-Old Man

Soft-tissue sarcoma is the most prevalent primary malignant cardiac tumor. This sarcoma usually presents with cardiac manifestations secondary to local obstruction or arrhythmias; very rarely does it present with initial symptoms of distant metastasis. We discuss the unusual case of an 18-year-old man who emergently presented with acute-on-chronic back pain. Imaging revealed a lesion on the 12th thoracic vertebra and a large mass arising from the left atrium. The cardiac mass was resected, and immunohistochemical analysis revealed it to be a pleomorphic sarcoma that had metastasized to the spine. The patient died 2 years later of diffuse metastases. In addition to the patient''s case, we discuss the nature and treatment of cardiac sarcoma.Key words: Bone neoplasms/secondary, heart neoplasms/diagnosis/pathology/surgery, sarcoma/complications/diagnosis/surgeryPrimary cardiac tumors are rare and usually benign. Approximately 15% to 20% of cardiac neoplasms are malignant.^1,2 Chief among these is soft-tissue sarcoma, the second-most prevalent of all primary cardiac tumors.^3,4 The typical presentation of cardiac sarcoma results from an anatomic obstruction of blood flow. Initial presentation in the form of occult distant metastasis is highly unusual, and only sporadic cases have been described.⁵ We report the case of a young man in whom a metastatic cardiac sarcoma presented as back pain, and we discuss the nature and treatment of these neoplasms.     

Sarcoma epitelioide del ilíaco derecho imitando una sacroilitis

Epithelioid sarcoma represents less than 1% of soft tissue sarcomas and has a high recurrence rate and strong ability to invade the regional lymphatic pathways. Classic epithelioid sarcoma has a predilection for young men and usually affects the distal extremities, while the proximal-type is characterized by different histological features and most commonly occurs in the proximal part of the extremities, trunk and pelvis. Intraosseous tumor development is rare.We describe a 47 year old patient with sacroiliac pain mimicking sacroiliitis. A computed tomography of the pelvis showed a destructive intraosseous lesion of the upper- right iliac. Percutaneous biopsy of the lesion was obtained and histological findings were typical of classic epithelioid sarcoma. To our knowledge, this is the second case of intraosseous epithelioid sarcoma described in the medical literature.     

Heart failure resulting from giant left atrial synovial sarcoma metastasis

Synovial sarcoma metastasis affecting the heart and infiltrating the mitral valve is a very rare pathology. We report the case of a 44-year-old male treated with chemotherapy for atypical synovial sarcoma of the oral mucosa who presented to our clinic after cardiac decompensation with a presumptive diagnosis of myxoma of the left atrium. A large necrotic tumour positive for CK 22, EMA, CD 99 and BCL-2 but negative for translocation in COBRA-FISH analysis by break-apart probe could be excised and revealed a very rare subtype of synovial sarcoma metastasis arising from the endocard of the left atrium. The tumour was resected and the mitral valve reconstructed through ring annuloplasty.     

Low-grade fibromyxoid sarcoma of the colon.

Low-grade fibromyxoid sarcoma is a recently recognised soft tissue neoplasm. It is rare and has a tendency to arise from deep soft tissue of the lower extremities. An origin from the colon has not been reported in the medical literature. We report the low-grade fibromyxoid sarcoma originating from the colon in a 43-year-old male patient, treated by right hemicolectomy and nephrectomy.     

Multiple cardiac metastases from Ewing's sarcoma

Ewing's sarcoma commonly arises from bone and sometimes from extraskeletal soft tissues. It commonly metastasizes to the lungs, bone, bone marrow and brain. Cardiac metastasis is extremely uncommon. The present report discusses a case of Ewing's sarcoma metastasizing to the heart at the time of initial presentation.     

Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumors in Bronchus

Ewing sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET), a member of the Ewing sarcoma family of tumors, is a malignant soft tissue tumor with small undifferentiated neuroectodermal cells. Primary trachea-bronchial ES/pPNET is very rare. The most common pulmonary ES is due to a metastasis. We describe a case of ES/pPNET which originated in the left basal trunk bronchus. The patient was a 30-year-old male, presenting with irritable cough and fever for 10 days. A tumor of 60 mm in diameter was found in the left basal trunk bronchus, extending to the left lower lobe. No distant metastases were detected. Histopathological examination revealed a malignancy of ES/pPNET with a diffuse proliferation of round cells, a Flexner-Wintersteiner rosette formation and positive staining for CD99. The patient was successfully treated with a combination of left lower lobectomy and adjuvant chemotherapy and has remained disease-free for approximately 18 months at follow-up. This case highlights that ES/pPNET should be considered as a differential diagnosis in cases of trachea-bronchial tumors.     

Myeloid sarcoma with megakaryoblastic differentiation presenting as a breast mass

Myeloid sarcoma is an extramedullary tumor that consists of myeloblasts or immature myeloid cells. The neoplasm can occur in any part of the body, including the bone, periosteum, lymph nodes, skin, and soft tissue and they may occur de novo or in association with acute myeloid leukemia, myeloproliferative neoplasms and myelodysplastic syndromes. Most cases display a myelomonocytic or pure monoblastic morphology. Tumors with megakaryoblastic differentiation are extremely uncommon and may occur in association with transformation of a myeloproliferative disorder. Myeloid sarcoma presenting as a breast mass is very rare and diagnostically challenging. We report a case of myeloid sarcoma with megakaryoblastic differentiation in the breast of a patient with history of essential thrombocythemia. The mass was composed of undifferentiated pleomorphic malignant cells in trabecular cords and nests with many scattered giant malignant cells and brisk abnormal mitoses. On immunohistochemistry, the neoplastic cells were positive for CD61, CD31, CD34, Factor VIII and CD43 which confirmed the diagnosis. To our knowledge, this is the first report of myeloid sarcoma with megakaryoblastic morphology occurring in the breast. Here we discuss the clinicopathologic features of this rare entity and the challenges involved in making this difficult diagnosis.     

原发性肺肉瘤的诊治进展

原发性肺肉瘤为发生于肺部少见的软组织恶性肿瘤,极易误诊,常规治疗首选手术.本文对该病的临床特点、影像学特征、病理学特点、支气管镜检查及治疗进展作了综述.