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Objective@#To investigate the clinicpathological and molecular features of Erdheim-Chester disease (ECD) as well langerhans cell histiocytosis (LCH).@*Methods@#The clinical, histopathological, molecular findings, immunophenotype, treatment and prognosis in 4 cases of ECD combined LCH were evaluated from February 2015 to September 2018 with review of the relevant literature.@*Results@#2 cases were male, and 2 were female, aged from 7-55 years. Microscopically, there were two different areas, in the first area, the lesions were composed of foamy histiocytes, spindle-shaped fibroblasts, scattered multinucleated giant cells. Lymphocytes, plasma cells, and giant cells were also found. In the other, the lesions were composed of histiocytes with obvious nuclear groove, associated with a variable number of eosinophils, lymphocytes and plasma cells. Immunephenotype, In the second area, histiocytes were positive for CD1a (4/4), S-100 (4/4),CD207/Langerin (4/4), cyclin D1(4/4), and in the two different area, the histiocytes were positive for CD68, CD163, Braf. Ki-67 positive index 1%-10% BRAF V600E gene mutation was detected in three cases.@*Conclusion@#ECD combined LCH was a very rare histiocytosis tumor and its correct diagnosis relies on histopathologic features, immunohistochemical staining, and BRAF V600E gene detection.
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Objective@#To investigate the clinicopathological features, differential diagnosis, and genetic alteration of Langerhans cell sarcoma (LCS).@*Methods@#Four cases of LCS were collected from Fujian Provincial Hospital and Fuzhou General Hospital of Nanjing Military Command of PLA from July 2013 to January 2017. Clinicopathological features and immunophenotype were retrospectively reviewed in four LCS cases combined with genetic mutation analysis of BRAF and ALK.@*Results@#Four cases included 2 women and 2 men with ages from 42 to 79 years (median=59.3 years). The size of the tumors ranged from 2.5-7.8 cm. Histologically, at the low power field, the tumors consisted of highly cellular proliferation in fascicules, whirlpool and diffuse sheets arrangement. The tumor cells were kidney-or horseshoe-shaped to round epithelioid cells or enlarged spindle cells. The neoplastic cells showed cytological atypia, hyperchromatic nuclei with prominent 1 to 2 nucleoli. Multinucleated giant cells were also found. Mitotic activity was approximately (50-70) mitoses/10 HPF. Immunohistochemically, the tumor cells were positive for S-100 protein (4/4), SOX10(3/4), Langerin/CD207(4/4), CD1a(3/4), CD68(3/4), CD163(3/4), and INI-1(4/4). Ki-67 index was 30%-80%. Gene mutation analysis showed that one case had BRAF V600E mutation but none had ALK gene alteration.@*Conclusions@#LCS is a rare tumor with highly malignant potential and distinct morphologic features.The primary treatment for LCS is completely surgical excision and chemotherapy. The prognosis is generally poor.
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Objective@#To observe the deposition of complement C3d at different development stages in human normal organs and tissues, and investigate the significance of its deposition.@*Methods@#Using immunohistochemical methods, the deposition of C3d was detected at different development stages of 60 normal human organs and tissue specimens and double staining was performed in some specimens. Ninty-five cases of other organs or tissues were collected as control group.@*Results@#In 50 of 60 livers, it was observed the deposition of C3d in Glisson′s capsule and periportal sheath, with irregular linear network-like disposition surrounding the portal sheath. In different age groups, the expression of C3d was more beyond the 20 year-old group than 3 to 20 year-old group. There wasn′t any expression of C3d under 3-year-old group. Under the immuning electron micrograph, C3d depositing at the Glisson′s capsule was observed, without immuning compounding. Thirty in 40 spleens, deposition of C3d in capsules, arteries of lymphatic sheath, follicles in the spleen was observed.@*Conclusions@#The deposition of C3d in Glisson′s capsule, splenic trabeculae, fibrous sheath, endarterium of liver and spleen arterioles, within normal human tissues from patients elder than 3 years, are osmosis/immunogenic deposition. The deposition of C3d is a normal physiological phenomenon, and treatment of the deposition of C3d should be avoided, as it is an immune complex or immuning reaction phenomenon.
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Purpose To investigate the clinicopathological features,diagnosis and differential diagnosis of myxoid synovial sarcoma (MSS).Methods Clinicopathological changes and immunophenotype were retrospectively evaluated in two MSS cases collected from Fujian Provincial Hospital,conbined with genetic mutation analysis.The relevant literatures were reviewed to explore its clinical and pathological features of this tumor.Restilts The two cases,one man and one woman,aged 71 and 15years,respectively.Tumor was located in the left down abdomen in case 1,and left frontal temporal lobe in case 2.Histopathologically,at low magnification in case 1,the tumor was nodular,which was made up of areas of hypercellularity and hypocellularity.In some areas of hypocellularity,the tumor cells were arranged in fascicular,story-form,sheet arrangements with mucoid degeneration.In hypercellularity area,the tumor cells were arranged in fascicular,fish bone-liked arrangenents.At low magnification in case 2,the tumor was nodular,which was made up of areas of hypercellularity and hypocellularity.In hypocellularity area,the tumor cells were arranged in net-like,sheet arrangements,and fascicular,sheet arrangements in case 2.In some area,the tumor cells were epithelioid with cluster distribution,without infringing brain tissue.Immunohistochemically,the tumor cells were diffusely positive for BCL-2,vimentin,and α-SMA and EMA were partially positive,while CD34,CD57,S-100,CD117,PLAP were negative.However,in case 2,only BCL-2 was positive,and MyoD1,GFAP,Olig-2,EMA,Syn,CD99,CgA,S-100,Myogenin,STAT6,CD34,desmin and α-SMA were negative.Molecular detection SYT-SSX fusion gene was detected in both cases.Conclusion MSS is a rare malignancy of soft tissue.The diagnosis of MSS depends on molecular pathology.The clinical and pathological findings are different from mucinous fibrosarcoma and solitary fibrous tumor.The treatment is surgical resection,combined with radiotherapy,with poor prognosis.
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<p><b>OBJECTIVE</b>To study the clinicopathologic features, diagnosis and differential diagnosis of extraskeletal myxoid chondrosarcoma (EMC).</p><p><b>METHODS</b>The clinical and pathologic features of 7 cases of EMC encountered in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command during the period of 2005 to 2015 were analyzed. Immunohistochemical study and PAS staining were carried out. Relevant literature was reviewed.</p><p><b>RESULTS</b>The male-to-female ratio was 6 to 1. The age of patients ranged from 21 to 50 years (median = 36 years). The maximum tumor dimension ranged from 2.5 to 15.0 cm (mean = 8.4 cm). The sites of involvement included left neck, right shoulder, left thigh, right thigh, right upper arm and abdomen. Most patients presented with painless lumps. Histologically, all cases showed similar features. Low-power examination showed a nodular or lobulated architecture, with intervening fibrous septa and myxoid matrix in the background. The tumor cells were arranged in cords or tufted clusters. They were spindly to epithelioid / rhabdoid (plasmacytoid) in shape, with eosinophilic to sometimes vacuolated cytoplasm. Intracytoplasmic eosinophilic inclusion bodies and coagulative necrosis were focally seen. Mitotic figures were rare (less than 2 per 10 high-power fields). Immunohistochemical study showed that the tumor cells were positive for vimentin (7/7) and INI1 (7/7). They were focally positive for CKpan (2/7), p63 (3/7), CD99 (3/7), S-100 protein (1/7) and synaptophysin (2/7). Ki-67 proliferation index ranged from 10% to 40%. The tumor cells were negative for α-smooth muscle actin, desmin, myoD1, CD34 and CD117. The cytoplasm of the tumor cells was positive for PAS. EWSR1 gene signal was detected in 5 cases.</p><p><b>CONCLUSIONS</b>EMC is a rare malignant mesenchymal tumor. Arrival at correct diagnosis relies on morphologic examination and immunohistochemistry. Molecular pathology is helpful when necessary. The primary treatment modality for EMC is complete surgical excision and the prognosis is satisfactory.</p>
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , Chondrosarcoma , Diagnosis , Pathology , Diagnosis, Differential , Immunohistochemistry , Neoplasms, Connective and Soft Tissue , Diagnosis , Pathology , S100 Proteins , Synaptophysin , VimentinABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinicopathologic features, diagnosis, differential diagnosis of multiple Rosai-Dorfman disease (RDD).</p><p><b>METHODS</b>Seven cases of multiple RDD were treated in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command of Chinese PLA. The disease was analyzed, focusing on the process of diagnosis, the treatment and follow-up. Histopathology, immunohistochemical profiles and relative literature were reviewed to reveal the characteristics of this disease.</p><p><b>RESULTS</b>The seven cases, occurred in 3 women and 4 men from 18 to 60 years of age (median 45.71 years), with masses measured of 0.8-6.0 cm (average size of 3.0 cm). Masses located in, left subcutaneous arm, thyroid, paratrachea, left maxilla, right subcutaneous cheek, left subcutaneous chest wall, right subcutaneous inguina, bilateral subcutaneous neck, right tibia, right thigh skin, right frontal lobe of brain, cerebral parafalx and bilateral lymph nodes of the neck, respectively. Among the cases, from the first case to the sixth case were extranodal tissue, and the seventh case was located in lymph nodes. Cases showed progressive increase of the mass. Histologically, all lesions of seven cases were similar with nodular structures presenting with alternating hyper- and hypo-cellular areas. The hypo-cellular areas revealed lymph-sinustoid structure characterized by variable numbers of large histiocytes, which had an abundant cytoplasm, pale to eosinophilic in appearance, phagocytozed intact lymphocytes or emperipolesis. While hyper-cellular areas revealed the infiltration of lymphocytes, plasma cells, neutrophils and numerous collagen fiber. Two cases also revealed the infiltration of lymphoid follicles. Immunohistochemically, the large histiocytes were strongly positive for S-100, CD163 and CD68 protein.</p><p><b>CONCLUSIONS</b>Multiple RDD is rare. In clinic and pathology, it needs to be differentiated from granulomatous diseases, IgG4-related sclerotic diseases, inflammatory myofibroblastic tumor, fibrohistiocytoma, Langerhans cell histiocytosis, and so on. The primary approach of treatment for multiple RDD is complete surgical excision and its prognosis is good.</p>