Sclerosing angiomatoid nodular transformation of the spleen,a rare cause for splenectomy: Two case reports

BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare benign disease of the spleen with unknown origin.Clinical symptoms are inhomogeneous,and suspicious splenic lesion often found incidentally,leading to splenectomy,as malignancy cannot securely be ruled out.Diagnosis is made histologically after resection.CASE SUMMARY Two cases of German,white,non-smoking,and non-drinking patients of normal weight are presented.The first one is a 26-year-old man without medical history who was exhibiting an undesired weight loss of 10 kg and recurring vomiting for about 18 mo.The second one is a 65-year-old woman with hypertension who had previously undergone gynecological surgery,suffering from a lasting feeling of abdominal fullness.Both showed radiologically an inhomogeneous splenic lesion leading to splenectomy approximately 6 and 9 wk after surgical presentation.Both diagnoses of SANT were made histologically.Follow-up went well,and both were treated according to the recommendation for asplenic patients.CONCLUSION SANT is a rare cause of splenectomy and an incidental histological finding.Further research should focus on clinical and radiological diagnosis of SANT as well as on treatment of patients with asymptomatic and small findings.     

卵巢硬化性间质瘤的CT诊断

目的 总结卵巢硬化性间质瘤（SST）的CT表现,以提高对其在CT影像学上的认识,掌握其诊断要点及主要鉴别诊断,降低误诊率.方法 分析3例经手术和病理证实的卵巢硬化性间质瘤CT表现及其特点.结果 3例均为单发,病灶与卵巢关系密切,病灶边界清晰,不侵犯周围临近器官,病灶密度多不均匀,多呈囊实性改变,动态增强病灶实性部分动脉期强化明显,静脉期继续强化,呈＂海绵状血管瘤样＂渐进性强化,囊性部分不强化,肿瘤壁多呈厚薄不均、结节状改变.结论 卵巢硬化性间质瘤在CT动态增强扫描时具有延迟强化特征性表现,肿瘤壁有结节样强化特点,在动态增强上具有一定的特征.     

Oral and Maxillofacial Sclerosing Epithelioid Fibroscarcoma: Report of Five Cases

Sclerosing epithelioid fibrosarcoma (SEF) has distinctive morphology and occurs mainly in deep soft tissue of adult extremities. Approximately 59 cases of SEF have been reported, with only 12 previously described in head and neck locations. Lesions involving the oral and maxillofacial region (OMFR) and intraosseous examples are rare. We present five cases of OMFRSEF. The OMF Pathology Department Registry was searched for cases coded from 1990 to the present as “SEF,” “fibrosarcoma not otherwise specified” or “neoplasm of uncertain histiogenesis.” Inclusion required OMFR location, an abundantly sclerotic sarcoma with epithelioid features, and lack of other phenotype by immunohistochemistry. Five cases of SEF included 3 males and 2 females. The age of the patients were: 19, 22, 35, 47 and 47 years. Tumor location included the infra-temporal fossa, buccal mucosa (recurrence extending into bone), anterior mandible (intraosseous primary, focally extending into soft tissue), and left parotid and submandibular gland (with metaplastic bone) regions. Tumor sizes ranged from 1.0 to 5.7 cm, median 3.5 cm. Histologically, the tumors were well delineated and multinodular, separated by fibrous septae. The spindled to primarily epithelioid tumor cells formed moderately cellular sheets and cords of irregularly contoured medium to large, round to oval, occasionally overlapping nuclei, indistinct nucleoli, wispy eosinophilic (retracting) cytoplasm, and distinctive cytoplasmic borders, embedded in osteoid-like stroma. Hemangiopericytoid (HPC-like) vessels were observed. Despite numerous apoptotic cells, mitoses were generally low; necrosis was present in two cases. Three tumors were graded as 2/3 and two 1/3. Immunohistochemically, the tumor cells were positive for vimentin, 1 case focally for CD34, whereas all cases were negative for S100 protein, keratins, EMA, desmin, and SMA. Wide or radical excision was performed with no adjuvant therapy. Follow-up revealed that 4 cases recurred at a range of 12–120 months. One case had no recurrent/residual disease at 3 months. Metastatic disease was present in 2 cases, to chest wall and lumbar/thoracic spine at 12 and 21 months, respectively. One patient died of disease complications at 15 months. OMFRSEF occur in adults in various locations, but with a common propensity to involve bone; there is recurrent potential and morbidity with higher grade lesions. The differential diagnosis for these tumors in this site includes sclerosing carcinoma, Ewing/PNET, osteosarcoma, osteoblastoma, and benign and malignant myoepithelial salivary gland tumors. The collagen, focal spindle cell features, HPC-like vasculature, and weak focal CD34 reactivity in one case might have raised a possible relationship between OMFRSEF and low grade malignant solitary fibrous tumor, but the intraosseous propensity, epithelioid features and relative lack of CD34 make this a distinctive entity.     

A fatal case of sclerosing mesenteritis

A 22-year-old patient was admitted because of abdominal pain and vomiting. Computed tomography diagnosed small intestinal malignancy. Ileal resection was performed, and the histological findings were consistent with sclerosing mesenteritis. The patient was treated with enteral nutrition, corticosteroids, azathioprine and methotrexate, but died 2 years later.     

Autosomal dominant type of endosteal hyperostosis with unusual manifestations of sclerosis of the jaw bones

We report three cases of autosomal dominant type endosteal hyperostosis which occurred in one Japanese family. A new pattern of sclerotic changes in the jaw bones is evident. In all members of the family there was a symmetrical thickening of the diaphyseal cortices of the long bones. The affected bones were only minimally widened and the epiphyses and metaphyses were spared. Endosteal sclerosis of the neurocranium was present with loss of the diploë. The sclerotic changes included enlargement and mottled sclerosis of both the maxilla and mandible, with multiple embedded teeth and odontomas. The ramus of the mandible was spared. Severe sclerosis of the jaw bones was present only in a 28-year-old woman. The 2-year-son showed only focal sclerosis in the mandible, and his grandmother had minimal changes in the skeleton.     

肺硬化性血管瘤43例临床病理分析

目的：探讨肺硬化性血管瘤（SHL）的临床病理特点和组织发生。方法：对43例经外科手术切除的SHL临床和病理资料进行回顾性分析和随访，15例进行TTF－1、EMA，vimentin,CK(AE1/AE3）、CK7、CK5／6、S－100、NSE、Syn,Caa,NF,CD34、Ⅷ因子和SMA免疫组化研究。结果：组织学以瘤细胞形成混合型结构为主要表现，23．3％有周围肺泡／和上皮浸润；立方细胞和多角形细胞共同表达TTF－1和EMA，AE1／AE1／AE3和CK7仅在立方细胞中表达，2例局部散在表达CgA；随访39例，随1例再发外余无转移和复发，结论：SHL可能起源皇幼稚的呼吸上皮，以乳突和实体结构形成为主者应与腺瘤和典型类癌鉴别。部分肿瘤有侵袭性生长的倾向。外科手术治疗宜行瘤切除非肿块剥离。     

Sclerosing pancreatitis showing rapidly progressive changes with recurrent mass formation

Summary Conclusion Sclerosing pancreatitis might develop repeatedly or might rapidly extend to the whole pancreas with recurrent mass formation. Background Nothing is known concerning course or development of sclerosing pancreatitis. Methods A 63-yr-old male was followed up for 2.5 yr. Results The patient was admitted because of a tumor in the body and tail of the pancreas. Serum pancreatic enzymes were transiently elevated, but tumor markers were all negative. Imaging studies showed a tumor 7 cm in size. The main pancreatic duct was normal in the head and obstructed at the body on endoscopic retrograde pancreatography (ERCP). The K-ras oncogene mutation was positive in pure pancreatic juice. Distal pancreatectomy was performed because pancreatic cancer was highly suspected. Pathological findings showed that the tumor was a densely fibrotic mass without malignant cells. Inflammatory cell infiltration was observed in the stroma. One year later, another mass 3 cm in size was noted in the remnant pancreatic head. ERCP revealed diffuse irregular narrowing of the main pancreatic duct, its branches, and the common bile duct. Liver dysfunction improved and an elevation of serum pancreatic enzymes subsided without any specific treatment, and the mass diminished in size. The patterns of various imaging studies on the second tumor were the same as those of the previous resected mass. Corticosteroid was not administered.     

肺硬化性血管瘤CT分析(附4例报告)

目的：分析肺硬化性血管瘤CT形态学及强化扫描表现特点。方法：1996－2001年经CT检查并经手术、病理证实的4例肺硬化性血管瘤。4例病灶均经薄层（3.0mm)CT平扫和对病灶中心层面进行延迟7min动态CDT增强扫描。分析病灶形态学表现，计算病灶平扫CT值与增强后病灶内部最大强化CT值差，简称CT增强值。结果：4例病灶形态均表现圆形、边缘光滑、无分叶、无毛刺和卫星病灶，病灶大小均在3.0cm以内。增强扫描4例病灶均呈明显强化，CT增强值为70－130HU，延迟扫描6min病灶强化仍较明显。结论：病灶呈圆形、边缘光滑、无分叶、无毛刺和卫星病灶，病灶大小多在3.0c,以内，增强扫描病灶呈明显强化，强化持续时间长为肺硬化性血管瘤CT表现特点。     

硬化性上皮样纤维肉瘤1例报道并文献复习

目的探讨硬化性上皮样纤维肉瘤的临床病理特点及鉴别诊断要点。方法对1例硬化性上皮样纤维肉瘤进行临床病理分析及免疫组化研究。结果肿物位于肩背部深肌层，鱼肉样，灰白色；镜下瘤细胞形态基本一致，呈圆形、卵圆形，胞质空而透亮，似上皮细胞，间质呈现明显的玻璃样变，免疫组化，vimentin( )。结论本瘤的特征是在大量硬化性间质的背景下出现上皮样肿瘤细胞，免疫组化仅vimentin( )。     

Three cases of sclerosing lipogranuloma: an immunohistochemical study

Sclerosing lipogranuloma (SLG) is a rare disease that occurs in the male genital region. We report here three cases of SLG, including two of Y-shaped lesions in the penile base forming an intrascrotal mass, as well as a patient with a mass in the epidydimal region. All three lesions histologically exhibited formation of multiple granulomas consisting of multinucleated giant cells and epithelioid cells, in the fibroadipose tissue or around the epidydimis, in association with eosinophil infiltration. Membranocystic changes were found within the cytoplasm of multinucleated cells. In the two patients with scrotal lesions, membranocystic changes were positive for diastase-PAS reaction and on Sudan black B staining in dewaxed sections. CD68 staining was strongly positive in multinucleated giant cells and epithelioid cells. Most of the lymphocytes infiltrating the lesions were T cells associated with some S-100-positive dendritic cells. T-cell mediated immune reaction appears to be important in the histogenesis of SLG. The histogenesis is generally considered to be a foreign-body reaction to degenerated or damaged fatty tissue or lipids, but no apparent causative factors can be identified in some reported cases. All three patients in the present study had a good clinical course after biopsy or surgical excision. We thus report three cases of SLG including an unusual case in the epidydimal region, with some considerations regarding the histogenesis of SLG.