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1.
A case of dedifferentiated leiomyosarcoma of the uterus was examined using immunohistochemistry. The tumor arose in the myometrium, and was a whitish large nodule with hemorrhage and necrosis. Histologically it was a well differentiated leiomyosarcoma with foci showing epithelioid pattern, and in part resembling malignant fibrous histiocytoma (MFH) and giant cell tumor (GCT). Additionally, small round neoplastic cells arranged in an alveolar manner, simulating alveolar rhabdomyosarcoma, were seen in some areas. Neoplastic cells in well differentiated areas expressed desmin, muscle-specific actin and LeuM1, whereas those in epithelioid and poorly differentiated areas lacked these antigens. Instead, tumor cells in epithelioid and small round cell areas were positive for keratin. Interestingly, most tumor cells in well differentiated, epithelioid and small round cell areas were also positive for MB1. However, tumor cells in GCT- and MFH-like areas reacted with none of the antibodies used. Ultrastructurally, some tumor cells possessed various amounts of microfilaments with or without dense patches, whereas others lacked them. These findings suggest that the divergent antigen expression was attributable to different levels of differentiation, and that poorly differentiated components had lost their native features.  相似文献   

2.
A case of dedifferentiated leiomyosarcoma of the uterus was examined using immunohistochemistry. The tumor arose in the myometrium, and was a whitish large nodule with hemorrhage and necrosis. Histologically it was a well differentiated leiomyosarcoma with foci showing epithelioid pattern, and in part resembling malignant fibrous histiocytoma (MFH) and giant cell tumor (GCT). Additionally, small round neoplastic cells arranged in an alveolar manner, simulating alveolar rhabdomyosarcoma, were seen in some areas. Neoplastic cells in well differentiated areas expressed desmin, muscle-specific actin and Leu Ml, whereas those in epithelioid and poorly differentiated areas lacked these antigens. Instead, tumor cells in epithelioid and small round cell areas were positive for keratin. Interestingly, most tumor cells in well differentiated, epithelioid and small round cell areas were also positive for MB1. However, tumor cells in GCT-and MFH-like areas reacted with none of the antibodies used. Ultrastructurally, some tumor cells possessed various amounts of microfilaments with or without dense patches, whereas others lacked them. These findings suggest that the divergent antigen expression was attributable to different levels of differentiation, and that poorly differentiated components had lost their native features. Acta Pathol Jpn 41: 466–472, 1991.  相似文献   

3.
We describe a rare case of gliosarcoma with primitive neuroectodermal, osseous, cartilage and adipocyte differentiation. A 57-year-old man experienced a month history of headache, nausea and vomiting. Worse yet, the headache has become more severe for the past 6 days. Magnetic resonance (MR) images disclosed a lesion with operative indications located in the right frontal lobe. Then the tumor was macroscopically totally removed. Histologically, the tumor showed two kinds of components. One kind of the tumor cells appeared typical astrocytic tumor cells with anaplastic appearance. The other kind of the tumor cells appeared sheets of small round hyperchromatic cells, which presented a kind of pancreatic neuroendocrine tumor (PNET)-like structure. These sheets of small round cells were surrounded by a large number of relative-sparse-spindle cells. Multiple separate distinct areas of adipose tissue, osteoid matrix laid down and cartilage tissue were also identified. Immunohistochemically, a portion of typical astrocytic tumor cells and some small round hyperchromatic cells showed GFAP positivity. Small round hyperchromatic cells were positive for S-100, Fli-1, Nestin, MAP-2 and Syn. A large amount of relative sparse spindle cells (sarcomatous areas) were positive for vimentin. In addition, reticulin staining demonstrated expression of reticular fibers in relative-sparse-spindle cells areas but not in the astrocytic tumor cells and small round hyperchromatic cells areas. Molecular cytogenetic analyses demonstrated PTEN allele loss and no evidence of amplification of EGFR in both the astrocytic tumor cells, PNET-like structure and sparse spindle cells areas. These data suggest that this tumor was a gliosarcoma with primitive neuroectodermal, osseous, cartilage and adipocyte differentiation. To our knowledge, this is a rare gliosarcoma , reporting our additional new case would add to the better understanding of this tumor.  相似文献   

4.
We report a case of high‐grade endometrial stromal sarcoma with cytological and immunocytochemical findings. Cytologically, major tumor cells showed round‐to‐short spindle shapes with round‐ to oval‐shaped nuclei and moderately abundant delicate cytoplasm. Tumor cells with tapered shapes and eccentric nuclei were also observed. A few spindle cells having enlarged cigar‐shaped nuclei with conspicuous nucleoli and delicate wispy cytoplasm, which resembled leiomyosarcoma, were intermingled. One rhabdomyoblast cell with both α‐sarcomeric muscle actin and myoglobin was also observed. Most of the tumor cells, including the leiomyosarcomatous spindle cells, were positive for CD10, and negative for desmin and h‐caldesmon. Accordingly, when relatively monotonous round‐to‐short spindle tumor cells and taper‐shaped tumor cells are observed in the female genital tract, high‐grade endometrial stromal sarcoma should be considered in the differential diagnosis. Immunocytochemistry contributed to the correct diagnosis. This case was high‐grade endometrial stromal sarcoma with smooth muscle and skeletal muscle differentiation. Diagn. Cytopathol. 2010. © 2010 Wiley‐Liss, Inc.  相似文献   

5.
An unusual case of malignant peripheral nerve sheath tumor (MPNST) arising in the posterior mediastinum of a 59-year-old man is reported. Histopathologically, the tumor showed an admixture of a dense proliferation of small round cells resembling a primitive neuroectodermal tumor (PNET) and a pleomorphic spindle cell sarcomatous area. Abortive rosettes, primitive neural tube-like structures, and a few glandular structures were found in the small round cell area. Small round cells were immunoreactive for neural cell adhesion molecule and synaptophysin, but were not immunoreactive for MIC2 and neuron-specific enolase. Pleomorphic spindle cells were occasionally arranged in a storiform pattern and were diffusely immunoreactive for S-100 protein. The MPNST of small round cell type is distinguishable from PNET by its negative immunoreactivity for MIC2, and the present tumor is assumed to be derived from primitive neuroectodermal cells in the peripheral nerve capable of bidirectional (neuron and Schwann cell) differentiation.  相似文献   

6.
A carcinoid tumor of the thymus combined with thymoma in a 62-year-old man is described. The mediastinal tumor had been present for 13 years and was associated with pure red cell aplasia. Carcinoid tumor occupied the central two-thirds of the tumor, consisting of nests and trabeculae of monotonous round cells, which ultrastructurally showed many intracytoplasmic dense-core granules. Typical spindle cell type thymoma surrounded the carcinoid area. Clinico-pathologic findings of this unique case suggested that the carcinoid tumor developed within a preexisting thymoma, illustrating a possibility of neuroendocrine differentiation of thymic epithelial cells.  相似文献   

7.
A 38-year-old woman was operated on to remove a large tumor that replaced the left adrenal gland. The tumor was encapsulated and showed small areas typical of pheochromocytoma, and spindle cell or undifferentiated round cell sarcoma in most areas. Metastases of primitive round cell appearance were operated from the abdominal cavity and abdominal wall shortly after the initial surgery. Eighteen months after the first operation, the patient was alive with metastases in liver and retroperitoneal space. The pheochromocytomalike component showed a typical ultrastructural and immunohistochemical profile of pheochromocytoma and was positive for neurofilaments, synaptophy-sin, neuron-specific enolase, and S-100 protein in the sustentacular cells. The sarcomatous areas showed fibroblastoid spindle cells that were often surrounded by a basal lamina. Immunohisto-chemistry revealed S-100 protein positivity in many spindle cells, but markers of pheochromocytoma or epithelial differentiation were absent. The metastases lacked all markers except for vimentin, and the cells were undifferentiated by electron microscopy. These findings suggest that the neoplasm was a compound tumor with a typical pheochromocytoma component and a sarcoma resembling a malignant schwannoma. Neoplastic proliferation of the S-100 protein-positive Schwann-cell-like sustentacular cells of the pheochromocytoma would be an explanation for the genesis of this sarcoma associated with pheochromocytoma.  相似文献   

8.
A 38-year-old woman was operated on to remove a large tumor that replaced the left adrenal gland. The tumor was encapsulated and showed small areas typical of pheochromocytoma, and spindle cell or undifferentiated round cell sarcoma in most areas. Metastases of primitive round cell appearance were operated from the abdominal cavity and abdominal wall shortly after the initial surgery. Eighteen months after the first operation, the patient was alive with metastases in liver and retroperitoneal space. The pheochromocytomalike component showed a typical ultrastructural and immunohistochemical profile of pheochromocytoma and was positive for neurofilaments, synaptophy-sin, neuron-specific enolase, and S-100 protein in the sustentacular cells. The sarcomatous areas showed fibroblastoid spindle cells that were often surrounded by a basal lamina. Immunohisto-chemistry revealed S-100 protein positivity in many spindle cells, but markers of pheochromocytoma or epithelial differentiation were absent. The metastases lacked all markers except for vimentin, and the cells were undifferentiated by electron microscopy. These findings suggest that the neoplasm was a compound tumor with a typical pheochromocytoma component and a sarcoma resembling a malignant schwannoma. Neoplastic proliferation of the S-100 protein-positive Schwann-cell-like sustentacular cells of the pheochromocytoma would be an explanation for the genesis of this sarcoma associated with pheochromocytoma.  相似文献   

9.
AIMS: Malignant thymic tumour histologically resembling a soft tissue sarcoma is extremely rare and defined as sarcomatoid carcinoma in the recent World Health Organization (WHO) classification. We report two such cases in which the tumour cells showed a prominent rhabdomyoblastic differentiation and analyse whether these tumours retain an epithelial nature at least in part. METHODS AND RESULTS: One tumour occurred in a 51-year-old man (Case 1) and the other in a 40-year-old woman (Case 2). Microscopically, both tumours consisted essentially of two types of tumour cells: spindle and large round cells, with no apparent epithelial components. Osteosarcomatous small foci were also found in Case 2. Immunohistochemically, desmin and muscle-specific actin were positive in the majority of both types of tumour cells, whereas myogenin was predominant in the spindle cells and myoglobin in the large round cells. Some of both types of cells expressed cytokeratin with co-expression of myoglobin in the large round cells, but with no myogenin in the spindle cells. Some cytokeratin-positive spindle cells were also negative for desmin. Ultrastructural examination of a recurrent tumour in Case 2 revealed some epithelial features among the spindle cells. Cytogenetic study of the same tumour showed a complex abnormality including der(16)t(1;16)(q12;q12.1), an identical pattern previously reported in a case of thymic squamous cell carcinoma. CONCLUSIONS: The findings support the definition in the WHO classification of sarcomatoid carcinoma that includes purely sarcomatous tumour as in the present cases. Occurrence of this type of tumour may indicate a relationship between thymic epithelial cells and myoid cells and/or a potential for divergent differentiation in thymic epithelial tumours.  相似文献   

10.
Xp11.2 translocation/TFE3 rearrangement-associated renal cell carcinoma (RCC) and Xp11 translocation renal mesenchymal tumor are distinct tumor entity. To broaden the spectrum of Xp11 neoplasms, we investigated a novel tumor exhibiting morphologies overlapping Xp11.2 translocation/TFE3 rearrangement-associated RCC and the mesenchymal counterpart with melanocytic differentiation by immunohistochemistry, fluorescence in situ hybridization (FISH) and RNA sequencing, as well as literature review. Histologically, the tumor was composed of three different types of tumor cells, including a large proportion of clear cells, small round cells, and a few spindle cells, presenting a relatively clear border in the majority area. The nuclei of all tumor cells showed extensively and strong positive expressions of TFE3. Whereas, the clear cells positively expressed the RCC-related markers including PAX8, RCC marker and CD10, and negatively expressed HMB45; On the contrary, the small round cells and spindle cells positively expressed melanocytic marker HMB45, and negatively expressed PAX8, RCC marker and CD10. The ki67 index was higher in the small round cells and spindle cells than that in the clear cells. FISH revealed the rearrangement of TFE3 gene in all the three types of cells. The NONO-TFE3 fusion gene was detected in all tumor cells by RNA sequencing. This unique Xp11 translocation-associated neoplasm might represent a distinct entity overlapping Xp11 translocation RCC and the mesenchymal counterpart with melanocytic differentiation, broadening the spectrum of Xp11 neoplasms. The patient died of tumor recurrence and lung metastasis after seven months after the surgery suggesting those tumors have an unfavorable prognosis.  相似文献   

11.
Recently, primitive neuroectodermal tumors (PNETs) have been shown to cover a wide spectrum of small round cell sarcomas, probably including some Ewing's sarcomas (ESs) and extraskeletal Ewing's sarcomas (EESs), in addition to classical peripheral neuroepitheliomas (PNs). In studies of small cell sarcomas, we found a group of undifferentiated tumors resembling PNETs with some features of neuroectodermal differentiation, but possessing areas of relatively large, pleomorphic cells. To clarify the nature of these tumors and their relationship to PNETs, we examined the variety of histological, immunohistochemical and ultrastructural features of 11 small cell sarcomas. Five of these tumors were composed of uniform, small round cells and were classified as PNs because of the presence of definite Homer-Wright rosettes and fibrillary processes. The presence of well developed neurite-like processes containing neurosecretory granules and immunoreactivities for various neural markers suggested that these PNs showed more advanced neuronal differentiation. Two tumors, with the classical features of ES, showed no ultrastructural evidence of neuronal differentiation, although only gamma-gamma neuron-specific enolase (NSE) positivity was detected. Four undifferentiated tumors with atypical features, included in this study as an atypical PNET group, showed certain neuroectodermal characteristics, such as ganglion cell differentiation, perivascular pseudorosettes, and gamma-gamma NSE reactivity. It is concluded from this study that PNETs may include small round cell tumors showing different degrees of neuro-ectodermal differentiation and some histological variations.  相似文献   

12.
A case of malignant spindle cell tumor occurring in the pericardium is presented. The tumor arose from the pericardium of a 51-year-old Japanese woman with no history of exposure to asbestos. The tumor extended into the pericardial and left pleural cavities. The primary and metastatic tumors consisted of fusiform cells with frequent mitoses. Ultrastructurally, the tumor cells possessed a discontinuous external lamina, cytoplasmic processes, microfilaments and desmosomal intercellular junctions. Immunohistochemical examination showed that most tumor cells were positive for Leu 7, and several for S-100 and glial fibrillary acidic protein. Unexpectedly, most of the tumor cells also expressed keratin. These findings favor a diagnosis of sarcomatous mesothelioma with aberrant antigenic expression or heterogeneous differentiation of neoplastic cells.  相似文献   

13.
Intra-abdomlnal desmoplastic small cell tumor (DSCT) usually occurs In infants and young male adults. A case of DSCT occurring in a 60 year old female Is described. No other apparent primary origin was detected. A mesocolon tumor, measuring 23times12times10cm, was composed predominantly of round to spindle cells which showed epithelioid- and focally sarcomatous arrangements. Irnmunohlstochemically, the tumor cells showed perinuclear dot-like staining of CAM5.2, many cells expressed HHF35, and some cells contained vimentin, epithelial membrane antigen, desmln, alpha-smooth muscle actin, neuron-specific enolase, or Leu 7. Electron microscopic examination showed that the tumor cells had mesenchymal-fibroblastic features. The tumor had an aneuploid DNA content with high S-phase fraction. The patient, who was treated with adjuvant chemotherapy, was alive, having had three recurrences in 36 months. In the second and third recurrent lesions, increased cellular atypla and fascicular arrangements of spindle cells were observed. DSCT should be included in differential diagnoses of postmenopausal pelvic tumors which show light-microscopically and immunohistochemically divergent phenotypes.  相似文献   

14.
Recently, primitive neuroectodermal tumors (PNETs) have been shown to cover a wide spectrum of small round cell sarcomas, probably including some Ewing's sarcomas (ESs) and extraskeletal Ewing's sarcomas (EESs), in addition to classical peripheral neuroepitheliomas (PNs). In studies of small cell sarcomas, we found a group of undifferentiated tumors resembling PNETs with some features of neuroectodermal differentiation, but possessing areas of relatively large, pleomorphic cells. To clarify the nature of these tumors and their relationship to PNETs, we examined the variety of histological, immunohistochemical and ultra-structural features of 11 small cell sarcomas. Five of these tumors were composed of uniform, small round cells and were classified as PNs because of the presence of definite Homer-Wright rosettes and fibrillary processes. The presence of well developed neurite like processes containing neurosecretory granules and immunore-activities for various neural markers suggested that these PNs showed more advanced neuronal differentiation. Two tumors, with the classical features of ES, showed no ultrastructural evidence of neuronal differentiation, although only gamma gamma neuron specific enolase (NSE) positivity was detected. Four undifferentiatied tumors with atypical features, included in this study as an atypical PNET group, showed certain neuroectodermal characteristics, such as ganglion cell differentiation, perivascular pseudorosettes, and gamma gamma NSE reactivity. It is concluded from this study that PNETs may include small round cell tumors showing different degrees of neuroectodermal differentiation and some histological variations. Acta Pathol Jpn 41: 444–454, 1991.  相似文献   

15.
A 37-year-old Japanese man with a solid and cystic pancreatic mass was referred to our hospital. Computed tomography revealed a well-demarcated solid and cystic mass measuring approximately 3.0 cm in diameter in the pancreatic body. The patient underwent middle segment pancreatectomy, and the retrieved tumor specimen was found to be a well-demarcated solid and cystic lesion measuring 3.0 × 3.0 cm. On histological examination, the cyst walls were found to be lined with a monolayer of non-atypical tall columnar epithelial cells. The solid areas surrounded the cystic ones and showed storiform proliferation of spindle cells that contained round, oval, or elongated nuclei and were present among abundant collagen fibers. The solid areas sent phylloid projections into the cystic spaces and the main pancreatic duct. The spindle cells were found to be diffusely positive for alpha-smooth muscle actin, desmin, and h-caldesmon on immunohistochemical analysis. Electron microscopy revealed that these cells possessed well-developed myofilaments with dense bodies, pinocytic vesicles, and basal lumina. Neither metastasis nor local invasion was detected. After the operation (4 years), tumor recurrence has not occurred. The main differential diagnoses of spindle cell tumors are leiomyomas, leiomyosarcomas, inflammatory myofibroblastic tumors, solitary fibrous tumors, extra-gastrointestinal stromal tumors, and schwannomas. However, the histological findings in the present case differed from those of these tumors. The present lesion is the first reported case of a primary pancreatic phyllodes tumor.  相似文献   

16.
We report an unusual case of spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid present in a 6-year-old boy. The tumor, located at both the left lobe and isthmus, was a circumscribed mass with slightly gritty whorled appearance. Microscopically, the lobulated, highly cellular, spindle cell neoplasm was arranged in intersecting bundles and fascicles separated by fibrous bands. Benign-appearing glands entrapped within fibrous bands and foci of squamous differentiation within spindle cells were observed. Immunohistochemically, the spindle cells were diffusely positive for cytokeratins, vimentin, and alpha-smooth muscle actin and patchily reactive for muscle-specific actin and epithelial membrane antigen, exhibiting myoepithelial differentiation. The spindle cells were also patchily immunopositive for p53 protein. Molecular genetic analysis revealed Ki-ras gene mutations at codons 13 (GGC(gly) to AGC(ser)) and 15 (GGC(gly) to AGC(ser)) on the same allele. Mutation of the p53 gene was not detected. This is the first report on Ki-ras oncogene mutations in a case of SETTLE.  相似文献   

17.
伴有神经内分泌分化的乳腺梭形细胞癌   总被引:6,自引:2,他引:4  
目的探讨乳腺伴有神经内分泌分化的梭形细胞癌的病理形态学和免疫表型特点及鉴别诊断。方法复习2500例乳腺癌切片,找出以梭形细胞占主要优势(〉80%)的癌5例,其中2例梭形细胞型导管内癌和3例梭形细胞型浸润癌。采用HE、阿辛蓝(AB)/PAS和网织染色,以及用癌胚抗原(CEA)、上皮膜抗原(EMA)、细胞角蛋白(CK7、3413E12、AE1/AE3)、神经元特异性烯醇化酶(NSE)、突触素、嗜铬蛋白(cg)A、Lue-7、波形蛋白,S-100、平滑肌肌动蛋白(SMA)、calponin、雌激素受体(ER)、孕激素受体(PR)、c—erbB-2、E-钙黏素、Ki-67、p53抗体进行免疫组织化学观察。其中4例有随访信息。结果患者平均年龄在68岁。镜下:5例癌细胞形态主要为长梭形的上皮样细胞,3例有少数胞质内空泡状细胞,4例可见散在AB阳性细胞。免疫组织化学5例均表达AE1/AE3、EMA、CEA、E-钙黏素和突触素,CK7有4例表达,NSE阳性3例,CgA和Lue7阳性2例,ER阳性4例,PR阳性2例,1例表达c-erbB-2,1例有灶状波形蛋白阳性。免疫组织化学结果显示2例梭形细胞型导管内癌和1例梭形细胞型浸润性癌是梭形细胞型的神经内分泌癌,另外2例梭形细胞型浸润性癌是伴有神经内分泌分化的化生性癌。随访3例存活(24~58个月),1例27个月内死亡。结论上皮样梭形细胞和细胞内黏液的出现是乳腺伴有神经内分泌分化癌的一个形态学特点。梭形细胞神经内分泌型导管内癌需要和普通导管增生及导管内乳头状瘤鉴别。梭形细胞型的神经内分泌癌和伴神经内分泌分化的梭形细胞浸润性癌需要与梭形细胞肌上皮肿瘤、恶性黑色素瘤及某些软组织肿瘤鉴别。  相似文献   

18.
Adenosarcoma is a rare uterine biphasic tumor composed of benign epithelial elements and a sarcomatous stroma. Although it is well described histologically, its cytological features are rarely mentioned in the literature. We describe a case of uterine adenosarcoma that was first detected by Papanicolau (Pap) smear. Numerous crowded clusters of spindle cells were present within a bloody background, as well as a few smaller, dyscohesive groups with cells showing high N:C ratio and oval to round nuclei with coarse chromatin and small nucleoli. A few nuclear grooves were identified. Adenosarcomas are rare lesions but should be considered in the differential diagnosis when spindled cells are noted in a pap smear.  相似文献   

19.
Because therapy for sarcoma often incorporates histologic subtype, grade, stage, and anatomic location, establishing a specific histologic subtype often is essential. To evaluate the effectiveness of fine-needle aspiration biopsy (FNAB) in histologic subtyping of soft tissue sarcomas, we retrospectively reviewed 73 consecutive aspirates from 67 patients, none of whom had a previously established sarcoma diagnosis. Sarcoma cases were subgrouped according to predominant cytomorphologic features: pleomorphic cell, 19; small round cell, 18; spindle cell, 18; myxoid, 10; epithelioid/polygonal cell, 7; 1 case of well-differentiated liposarcoma was analyzed separately. Ancillary studies were used for 25 cases. Among adequate specimens, 61 tumors were recognized as sarcoma. A specific and accurate histologic subtype was determined in 34 cases. Ancillary studies were most useful for histologic subtyping of small round cell and spindle cell sarcomas. Myxoid sarcomas were subtyped easily based solely on histomorphologic features. Pleomorphic cell and epithelioid/polygonal cell sarcomas were recognized easily as malignant but difficult to subtype by FNAB. With the exception of small round cell sarcomas, histologic subtyping of a sarcoma usually did not directly influence therapy. With meticulous attention to clinicopathologic features and ancillary techniques, many sarcomas, especially small round cell, spindle cell, and myxoid types, may be subtyped successfully by FNAB, within limitations.  相似文献   

20.
We report on the crush preparation findings of a case of "sarcomatoid" chordoma occurring in the sacral region of a 78-yr-old Chinese male. The smears showed clumps and small cords of polygonal tumor cells containing bubbly cytoplasm and round to oval nuclei. Focally, there were also aggregates of long filamentous spindle cells and stellate bizarre cells with marked nuclear pleomorphism. Occasional tumor cells were seen in association with dense amorphous material. Histologic examination of the excised specimen showed features of the so-called "sarcomatoid" chordoma which consisted of prominent foci of mitotically inactive spindle and pleomorphic cells, in addition to the conventional chordoma areas. An osteosarcoma-like pattern of probably metaplastic nature was also seen within the tumor. Immunohistochemical study showed that most tumor cells expressed cytokeratins. Ultrastructural examination revealed the characteristic rough endoplasmic reticulum-mitochondria complexes. While there are many spindle and pleomorphic cells seen in crush preparations, the distinction from other true high-grade malignancies is important. Recognition of these "pseudoanaplastic" cytologic features also helps to expand the morphologic spectrum of chordoma.  相似文献   

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