Undifferentiated embryonal sarcoma of the liver: case report and literature survey

Undifferentiated embryonal sarcoma (UES) of the liver was first identified as an independent clinicopathologic type of sarcoma in 1978. It is an uncommon hepatic tumor, of mesenchymal origin, usually observed in children, and cases in adults are rare: to our best knowledge, reports of only 51 cases have been published in the past 50 years. We present a case of UES of the liver in a previously healthy 22 year-old woman, admitted to our hospital due to a palpable mass in the right upper abdomen. On admission, laboratory studies showed mildly elevated aspartate aminotransferase, alkaline phosphatase, and γ-GPT. Hepatitis and tumor markers were negative. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) showed a large mass involving the right lobe and the medial segment of the liver, with compression of the bile duct. Right trisectionectomy with bile duct resection and reconstruction was performed. Microscopically, the tumor was composed of pleomorphic spindle cells in a myxoid stroma with focal staining of S-100 by immunohistochemistry. The histologic diagnosis was UES. Adjuvant therapy with vincristine, actinomycin-D, and cyclophosphamide was performed, and at 14 months of follow-up, the patient is alive without any evidence of recurrence. The clinical and histopathological features, as well as the therapeutic choices for adult UES, are described for this patient and in the literature of the past 50 years.     

Synovial sarcoma of the spine in the lumbar vertebral body: A rare case report

Rationale:Synovial sarcoma (SS) is a soft tissue neoplasm that rarely occurs in the vertebral body and should be considered in the differential diagnosis in patients with SS and vertebral lesions. SS often presents as a painless mass in the spine, which may undergo slow enlargement, resulting in sustained symptoms of neurologic deficit and pain. Due to the difficulty in differentiating between SS from other soft tissue tumors and metastatic tumors, careful histological confirmation is required for definite diagnosis. Furthermore, due to its malignancy, the appropriate treatment procedure for SS should be carefully considered.Patient concerns:A 56-year-old female patient had low back pain. Radiological examination revealed bony erosion of the L-2 vertebral body, and no soft tissue mass around the lumbar spine.Diagnosis:Histopathological and immunohistochemical examination revealed SS.Interventions:The initial treatment of posterior laminectomy decompression and percutaneous vertebro plasty (PVP) was performed, however, this initial treatment course was inappropriate, but she eventually underwent L-2 complete resection and internal fixation. After the second surgery, she was treated by external beam radiation therapy.Outcomes:operation radiotherapy was finally performed. No local recurrence in L-2 vertebral body or distant metastasis was found at 1-year follow up postoperation; the neurologic symptom gradually relieved, and no other symptom was noted. And no local recurrence in L-2 vertebral body and distant metastasis was found in 1 year follow up postoperation.Lessons:Solitary spinal SS is extremely rare. Early surgery for total resection and adjuvant radiotherapy/chemotherapy should be emphasized.     

Synovial haemangioma of the knee: a case report

Synovial haemangioma is a rare but important cause of knee symptoms, which, when undiagnosed, can lead to significant morbidity. Diagnosis is frequently difficult and delayed. We report on a case of synovial haemangioma, which demonstrates the difficulties inherent in diagnosis and the morbidity associated with diagnostic delay, in a young woman. Magnetic resonance imaging (MRI) is a useful tool for diagnosis, but detection on MRI can also be problematic, as shown by this case, demonstrating the need for greater awareness of this condition by both clinicians and radiologists. Arthroscopy is important in both the diagnosis and treatment of these lesions.     

Isolated pancreatic granulocytic sarcoma: a case report and review of the literature

Granulocytic sarcoma (GS) is an extramedullary tumor mass consisting of immature myeloid cells. Isolated pancreatic granulocyte sarcoma is extremely rare. We report a very unusual pancreatic granulocytic sarcoma in a patient without acute myeloid leukemia. The patient presented with acute epigastric pain because of splenic infarction due to a mass consisting of myeloblasts in the pancreatic tail. The patients underwent splenectomy and distal pancreatectomy. Pathology and immunohistochemistry suggested a GS....     

Nonleukemic myeloid sarcoma of the liver: a case report and review of literature

Myeloid sarcoma (MS) is a neoplasm of immature granulocytes, monocytes, or both involving extramedullary sites. MS with no evidence of leukemia (nonleukemic MS) is very rare and the initial diagnosis can be difficult. This report describes an unusual case of nonleukemic MS of the liver in a 16-year-old patient presenting as debilitating hepatomegaly. A liver biopsy revealed diffuse infiltration by neoplastic cells of myeloid lineage (CD68, myeloperoxidase). A bone marrow biopsy showed no evidence of medullary involvement. The patient subsequently developed heart failure. Autopsy revealed infiltration of most organs by neoplastic cells but failed to identify abnormal myeloid cells in bone marrow.     

Hypopharyngeal spindle cell lipoma: A case report and review of literature

Rationale:Spindle cell lipoma is a rare, uncommon type of benign lipomatous tumor, a distinct group of lipomas composed of mature adipocytes, uniform spindle cells, and multinucleated giant cells associated with ropey collagen. Immunohistochemically, spindle cell lipoma is characterized by the diffuse expression of CD34.Patient concerns:We present a rare case of a 56-year-old man who complained of vomiting out of a smooth and giant mass in the oral cavity provoked by an intra-abdominal pressure increase. Oral examination revealed an elongated mass protruding from the mouth. Computed tomography of the patient showed a mass from left pyriform to oral cavity, with 2.38 × 2.78 × 16.86 cm in size. The flexible fiberscope showed that the pedicle of the elongated mass originated from the posterior wall of the hypopharynx, corresponding to the left pyriform fossa.Diagnosis:Histopathologically, the tumor was mainly composed of hyperplastic adipocytes, admixed with small blood vessels, and scattered inside adipose tissue spindle cells. The immunohistochemical profile revealed positivity of spindle cells for CD34, negativity for S100, and low proliferation with Ki67, which confirmed the diagnosis of spindle cell lipoma and revealed its benign behavior.Interventions:The patient underwent hypopharyngeal mass resection using transoral suspension laryngoscopy.Outcomes:No recurrence was found after 5 months of follow-up.Lessons:Spindle cell lipoma is difficult to diagnose early because of slow growth and subtle symptomatology. This entity should be differentiated from several benign or malignant subtypes of lipomas, including liposarcomas. In this case, the spindle cell lipoma is large and originates from the hypopharynx, which is a rare entity and presents with atypical symptoms. This case gave rise to further studies on the clinical and pathologic characteristics of this tumor in the future.     

原发性肺滑膜肉瘤诊断与治疗附1例报告及文献复习

目的 通过探讨原发性肺滑膜肉瘤的临床症状、影像学表现、病理学及免疫组化,提高对本病的认识.方法 报告近期呼吸科确诊1例原发性肺滑膜肉瘤患者,同时对既往报道18例患者进行回顾性研究,分析其临床表现、影像学表现、病理学特征及免疫组化特点.结果 临床表现为咳嗽、咳痰或胸痛、胸闷等,影像学表现为肺部肿块影,边缘清楚或呈分叶状,病灶密度不均匀,增强扫描呈不均匀强化,可伴纵隔淋巴结肿大及胸腔积液,病理学检查提示为梭形细胞恶性肿瘤,瘤细胞呈流水状排列,核分裂象易见,免疫组化EMA、Bcl-2、Vimentin等阳性.结论 原发性肺滑膜肉瘤临床表现及影像学表现上无特异性,不易与其他肺或胸膜原发性肉瘤相鉴别,需要结合病理及免疫组化结果进行诊断.     

Myeloid sarcoma of the pancreas: A case report and literature review

Introduction:Myeloid sarcoma (MS) is an extramedullary mass, consisting of myeloid blasts with or without maturation, which efface the normal tissue architecture. It occurs mainly in lymph nodes, skin and soft tissue, testis, bone, peritoneum, and gastrointestinal tract, but rarely in the pancreas. Because their clinical courses, treatments, and prognoses are quite different, it is crucially important to distinguish between MS and pancreatic cancer.Patient concerns:We herein report a rare case of acute myeloid leukemia (AML) which presented with a pancreatic mass that mimicked pancreatic cancer.Diagnosis: The diagnosis of MS was established based on immunohistochemical (IHC) analysis and bone marrow examination which revealed neoplastic cells with CD34+/CD117+.Interventions:The patient was actively treated with chemotherapy.Outcomes:After 4 cycles of chemotherapy, the lesion in pancreas was significantly reduced, and the patient is still receiving further chemotherapy.Conclusion:When we encounter a patient suspected of pancreatic cancer with blood cell abnormalities and no significant increase in carbohydrate antigen 19-9 (CA19-9), we need to be aware of the possibility of pancreatic MS. Preoperative pathological biopsy and IHC are indispensable. Misdiagnosis is common if we rely solely on imaging.     

Aggressive Kaposi's sarcoma in a 6-month-old African infant: case report and review of the literature

Kaposi's sarcoma (KS), known to exist in Africa for a century now, was rare in children and unknown in the newborn. With the onset of the HIV/AIDS epidemic, a more aggressive, disseminated type of KS (AKS) was recognized. Recently KS was diagnosed in a 6-month-old infant in Tanzania. Data support the notion that HHSV8 infectivity can be potentiated with HIV infection and thus produce multiple lesions in different anatomical sites early in life. Furthermore, the available evidence would suggest a nonsexual route of HHSV8 infection, possibly from mother to fetus.     

Cutaneous langerhans cell sarcoma: a case report and review of the literature

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that occurs in lymph nodes, liver, skin, spleen, lung, and bone. We report a case of LCS in a 47-year-old man with a 6-month history of scalp mass and cervical lymphadenopathy. Clinical and pathologic data were available. A histologic examination demonstrated a proliferation of cells with malignant cytologic features. Because of its poorly differentiated morphologic features, hematologic and nonhematologic entities were ruled out by immunohistochemical screening with a broad panel of antibodies. Ultrastructural studies demonstrating Birbeck granules and consistent expression of CD1a, S-100 protein, and langerin by immunohistochemistry were helpful in identifying the Langerhans cell origin.     

Kaposi's sarcoma in association with Behcet's disease: case report and literature review

OBJECTIVES: To describe a case of Behcet's uveitis associated with Kaposi's sarcoma occurring simultaneously in a patient and to review the literature on iatrogenic Kaposi's sarcoma. METHODS: We describe the case of a 44-year-old Moroccan man, who developed a Kaposi's sarcoma 8 months after immunosuppressive therapy for ocular Beh?et's disease. He was treated with corticosteroids and cyclophosphamide (Exdoxan, Baxter) pulse for 6 months followed by oral azathioprine (Imurel, Glaxo Smith Kline). Literature searches were performed on iatrogenic Kaposi's sarcoma and other cases of such association and the potential pathogenic mechanisms involved. RESULTS: Iatrogenic Kaposi's sarcoma is widely reported to develop after renal transplantation during immunosuppressive therapy. Less commonly, Kaposi's sarcoma occurs in patients receiving long-term corticosteroids or immunosuppressive therapy for rheumatic diseases. It is considered to be induced by activation of latent human herpes virus 8. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with ocular Beh?et's disease. Interferon-alpha is of value for patients with both conditions. CONCLUSION: This case report underscores the relationship between environmental and infectious factors, drug-induced immunosuppression, and the development of Kaposi's sarcoma.     

硬金属致巨细胞间质性肺炎一例并文献复习

目的 提高对硬金属粉尘致巨细胞间质性肺炎的临床、胸部影像学和病理表现的认识.方法 对1例经胸腔镜肺活检证实的巨细胞间质性肺炎患者的硬金属粉尘职业史、临床、胸部x线、CT、病理资料并结合有关文献进行回顾性分析.结果 患者女,30岁,钨棒磨削工,接触含钴和钨等硬金属粉尘3年,咳嗽和活动后喘息40 d.肺功能检查示混合性通气功能障碍,弥散功能明显下降(D<,L>CO占预计值%为39%),X线胸片和高分辨率CT示两肺弥漫性分布的磨玻璃影和边界不清的小结节影.肺活检病理表现为巨细胞间质性肺炎,肺泡腔内可见巨噬细胞和大量的多核巨细胞聚集,位于肺泡腔内的多核巨细胞内可见被吞噬的炎症细胞;细支气管及周围肺泡腔可见慢性炎症细胞浸润和肺间质纤维化.结合患者的职业史及特征性病理改变,诊断为硬金属致巨细胞间质性肺炎和硬金属肺疾病.患者脱离接触金属粉尘工作后,经糖皮质激素治疗临床症状缓解,胸部异常阴影明显吸收.结论 巨细胞间质性肺炎是硬金属肺疾病的特征性病理改变.在间质性肺疾病的诊断和鉴别诊断中需重视患者的职业暴露史.     

原发性粒细胞肉瘤1例并文献分析

目的:分析原发性粒细胞肉瘤(GS)的临床特征包括疾病的发病率,诊断流程,预后和治疗策略等。方法:对我院近期内确诊的1例原发于卵巢的GS和近20年MEDLINE文献关于原发性GS的资料87例进行总结分析。结果:分析资料完整的88例患者,其中65例确诊为原发性GS后转化为急性非淋巴细胞白血病(AN-LL),其余23例未发生转化目前仍在随访中。淋巴结,皮肤,泌尿生殖系统和消化系统是GS最常见的髓外发生部位;经典的GS肿瘤细胞形态具有不同阶段髓细胞分化的特点,免疫组化示MPO,NCAE(氯化醋酸AS-D萘酚酯酶),CD43和CD68等阳性;37例患者初诊时被误诊,大多数误诊为恶性淋巴瘤。传统的ANLL化疗方案疗效明显优越于恶性淋巴瘤方案和单独放疗。结论:减少原发性GS向ANLL转化并延长生存期,关键在于早期确诊和早期采用ANLL方案全身化疗。     

Primary spindle cell sarcoma of gallbladder: An unusual case report and a literature review

Introduction:Primary spindle cell sarcoma of the gallbladder is a rare condition.Patient concerns:A 67-year-old woman was admitted to a local hospital with a chief complaint of abdominal pain in the right upper quadrant for the past 2 months.Diagnosis and intervention:Surgical resection was performed following the diagnosis of primary gallbladder sarcoma with local hepatic metastasis. Histological examination confirmed a diagnosis of primary spindle cell sarcoma and hepatic metastasis with simultaneous cholecystolithiasis.Outcomes:Adjuvant chemoradiation therapy was not performed because the patient refused treatment. Three months after the surgery, a relapsed lesion was diagnosed. The patient underwent transcatheter arterial chemoembolization.Conclusions:The disease should be differentially diagnosed from gallbladder carcinoma or carcinosarcoma with hepatic metastasis. An aggressive surgical approach should be based on a balance between the risk of surgery and the outcome.     

成人肝脏未分化胚胎性肉瘤1例

成人肝脏未分化胚胎性肉瘤,临床极其罕见,容易误诊.随着影像诊断及肝脏外科的迅速发展,对其发现可能会有所增加,作者就经治的1例此病并复习文献加以讨论,以提高对本病的诊治.     

Granulocytic sarcoma of the pancreas: a report of two cases and literature review

Granulocytic sarcomas (GS) are extramedullary tumour masses of immature myeloid cells, also known as chloroma and extramedullary myeloid cell tumour. These neoplasms usually occur simultaneously with, or follow the onset of, acute myeloid leukaemia (AML). Rarely, they are the first manifestation of AML. GS may also be the first sign of transformation to AML in patients with chronic myeloproliferative disorders and myelodysplastic syndromes. GS have been reported to occur in a variety of tissues, but presentation as an abdominal mass and, in particular, infiltration of the pancreas is rare. We report two cases of pancreatic GS, review the literature, and discuss recent insights into the basic biological properties of these rare tumours.     

Primary pulmonary synovial sarcoma, a rare primary lung neoplasm: two case reports and review of the current literature

Abstract:   This report describes the clinical, histological and immunohistochemical features of two patients with primary pulmonary synovial sarcoma in the context of the literature. Chest pain, cough, haemoptysis and an enlarging pleural-based mass are the main clinical manifestations. Diagnosis depends on identifying epithelioid or spindle cells microscopically and on immunohistochemistry showing positivity for cytokeratin and vimentin and epithelial membrane antigen stains. Surgical excision is the main treatment approach.     

Gastrointestinal Kaposi's sarcoma: Case report and review of the literature

Kaposi's sarcoma(KS) of the gastrointestinal tract is not an uncommon disease among individuals with acquired immunodeficiency syndrome(AIDS). The majority is asymptomatic, and for this reason, gastrointestinal KS(GI-KS) remains undiagnosed. With continued tumor growth, considerable variation in clinical presentation occurs including abdominal pain, nausea, vomiting, iron deficiency anemia(either chronic or frank gastrointestinal bleeding), and rarely mechanical obstruction alone or combined with bowel perforation. Endoscopy with biopsy allows for histological and immunohistochemical testing to confirm the diagnosis of GI-KS among those with clinical symptoms. In previous studies, dual treatment with highly active antiretroviral therapy and systemic chemotherapy have been associated with improved morbidity and mortality in individuals with visceral KS. Therefore, investigators have suggested performing screening endoscopies in select patients for early detection and treatment to improve outcome. In this review, we describe a 44 years old man with AIDS and cutaneous KS who presented for evaluation of postprandial abdominal pain, vomiting, and weight loss. On upper endoscopy, an extensive, infiltrative,circumferential, reddish mass involving the entire body and antrum of the stomach was seen. Histologic examination later revealed spindle cell proliferation, and confirmatory immunohistochemical testing revealed human herpes virus 8 latent nuclear antigen expression consistent with a diagnosis of gastric KS. Following this, we present a comprehensive review of literature on KS with emphasis on gastrointestinal tract involvement and management.