Journal of Pathology and Translational Medicine

Review

Dysembryoplastic Neuroepithelial Tumors: Yeon-Lim Suh; J Pathol Transl Med. 2015;49(6):438-449. Published online October 23, 2015; DOI: https://doi.org/10.4132/jptm.2015.10.05

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Dysembryoplastic neuroepithelial tumor (DNT) is a benign glioneuronal neoplasm that most commonly occurs in children and young adults and may present with medically intractable, chronic seizures. Radiologically, this tumor is characterized by a cortical topography and lack of mass effect or perilesional edema. Partial complex seizures are the most common presentation. Three histologic subtypes of DNTs have been described. Histologically, the recognition of a unique, specific glioneuronal element in brain tumor samples from patients with medically intractable, chronic epilepsy serves as a diagnostic feature for complex or simple DNT types. However, nonspecific DNT has diagnostic difficulty because its histology is indistinguishable from conventional gliomas and because a specific glioneuronal element and/or multinodularity are absent. This review will focus on the clinical, radiographic, histopathological, and immunohistochemical features as well as the molecular genetics of all three variants of DNTs. The histological and cytological differential diagnoses for this lesion, especially the nonspecific variant, will be discussed.

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Case Reports

Solitary Fibrous Tumor of the Conjunctiva with Heretofore Undescribed Pathologic Findings.: Na Rae Kim, Jae Y Ro, Kyung Hwan Shin, Hae Jung Paik, Jung Suk An, Seung Yeon Ha; Korean J Pathol. 2011;45(3):315-318.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.3.315

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Abstract PDF

A 37-year-old female presented with a conjunctival mass discovered 3 years prior. An excisional biopsy revealed a patternless proliferation of round and spindle-shaped cells with an eosinophilic fibrillary cytoplasm and vesicular nuclei with occasional inclusions. Psammoma bodies were arranged around the dilated irregularly-shaped vessels. Differential diagnoses included conjunctival solitary fibrous tumor (SFT), nevus, glomangioma, ectopic meningioma, and mesectodermal leiomyoma. The tumor cells were immunoreactive for CD34, CD99, bcl-2 and vimentin, and were negative for smooth muscle actin, desmin, glial fibrillary acidic protein, S-100 protein, epithelial membrane antigen, and human melanoma black-45. Ultrastructurally, the tumor cells had rough endoplasmic reticulum, free ribosomes, and scattered mitochondria without basal lamina or cellular junctions, which are features of fibroblasts. A diagnosis of SFT was rendered based on the light microscopic, immunohistochemical, and electron microscopic findings. We report here on the second case of a SFT arising in the conjunctiva, which clinically and histologically mimics conjunctival nevus, glomangioma, ectopic meningioma, and a hybrid neurogenic-myogenic tumor such as mesectodermal leiomyoma.

Citations

Citations to this article as recorded by

Meningeal Solitary Fibrous Tumors with Delayed Extracranial Metastasis
Nayoung Han, Hannah Kim, Soo Kee Min, Sun-Ha Paek, Chul-Kee Park, Seung-Hong Choi, U-Ri Chae, Sung-Hye Park
Journal of Pathology and Translational Medicine.2016; 50(2): 113. CrossRef

Spindle Cell Lipoma Involving the Larynx and Lateral Neck Space.: Young Ha Kim, Jung Hae Cho, Chan Kwan Jung, Dong Il Sun; Korean J Pathol. 2009;43(2):171-173.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.2.171

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Abstract PDF

Spindle cell lipoma (SCL) is a rare lipoma variant that account for approximately 1.5% of all adipocyte-origin tumors; SCL usually occurs on the posterior neck or shoulder. The histological characteristics of SCL include mature, univacuolar fat cells and fibroblast-like spindle cells in a matrix of collagen and mucoid material. It is important to note that spindle cell lipoma can be mistaken both clinically and histologically for liposarcoma. We report here on a rare case of SCL in a 48-year-old male, and the patient presented with a large right neck mass that involved the lateral neck space and larynx.

Citations

Citations to this article as recorded by

Spindle Cell/Pleomorphic Lipoma of the Oropharynx
Mi Jin Gu, Kyung Rak Sohn, Jun Ho Park
The Korean Journal of Pathology.2009; 43(6): 580. CrossRef

Original Articles

Microvessel Quantitation and Assessment of its Utility by CD34 Staining in Invasive Breast Carcinoma.: Hwa Sook Jeong, Ro Hyun Sung; Korean J Pathol. 1997;31(4):298-307.

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Abstract PDF: Tumor angiogenesis, the development of new blood vessels by tumor, is a widely observed phenomenon associated with the growth of human solid tumors. To investigate how tumor angiogenesis correlates with other prognostic features i.e. menopause status, tumor size, lymph node metastasis, mitosis, angioinvasion, estrogen receptor (ER), p53 protein expression, histologic grade and clinical stage, we counted microvessels by immunohistochemistry using antibody for CD34 antigen in 56 cases of invasive breast carcinoma (27 with and 29 without axillary lymph node metastases) and 20 cases of non-inflammatory benign breast lesion. CD34 antigen is expressed on the surface of hematopoietic progenitor cells and more sensitively expressed than factor VIII in vascular endothelial cells. Microvessel count (MVC) was performed at a single hot field of 200x magnification (0.74 mm2 per field). The results are summarized as follows; 1) The mean MVC of invasive carcinoma and benign breast lesion were 92.0+/-54.4 (range, 7-237) and 20.7+/-16.6 (range, 4-73), respectively (p<0.0001). 2) Although MVC had no correlation with all other prognostic factors i.e. menopause status, tumor size, lymph node metastasis, mitosis count, angioinvasion, ER, p53 protein expression, histologic grade, and clinical stage (p>0.05), MVC had a tendency to increase in tumors with axillary LN metastasis or without ER expression. 3) Without correlation with MVC, ER (+), angioinvasion (-) and higher histologic grade correlate to significantly higher mitosis count (p<0.0005). Also, angioinvasion correlate to a significantly higher histologic grade (p<0.05). In conclusion, angiogenesis is related to tumorigenesis, but MVC may not be related to other clinicopathologic factors.

CD34 Antigen Expression in Gastrointestinal Stromal Tumors.: Sun Hee Sung, Min Sun Cho, Woon Sup Han; Korean J Pathol. 1997;31(11):1166-1171.

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Abstract: Gastrointestinal stromal tumor (GIST) is known as considerable controversal tumor about it's histogenesis, differentiation and biologic behavior. It is traditionally regarded as smooth muscle tumor. To evaluate and clarify the origin of tumor, we performed immunohistochemical study of 23 cases of GIST on CD34 antigen, alpha-smooth muscle actin, S-100 protein, and compared the result with 4 cases of typical leiomyoma of GI tract. The results were as follows. CD34 antigen expression was noted in 21 cases (91.3%) of GIST, while typical leiomyoma was all negative. There were no difference of CD34 expression according to the biologic behavior. However, it's staining pattern was significantly different (p<0.05). Focal or multifocal expression was dominant in benign GIST (58.3%), while diffuse expression was dominant in malignant GIST (80%). Actin was expressed in 5 cases of benign GIST (38.5%) and 1 of malignant GIST (16.7%) focally. All typical leiomyoma showed diffuse strong positivity on alpha-smooth muscle actin. S-100 protein was expressed in 2 cases of benign GIST (16.7%) only. The pattern of CD34 expression was focal in the actin or S-100 protein positive cases. In conclusion CD34 antigen is useful marker in the separation of GIST, from typical smooth muscle tumor. Also it suggest that most GISTs are histogenetically primitive mesenchymal cell origin. However, CD34 expression was unrelated with biologic behavior of GIST.

Case Report

Progression of Dermatofibrosarcoma Proruberans to Malignant Fibrous Histiocytoma: Report of a case.: Hye Rim Park, Eun Ha Jung, Jin Hee Sohn; Korean J Pathol. 1997;31(12):1324-1327.

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Abstract PDF: A 52-year-old woman was admitted with a palpable mass on the abdominal wall. This tumor had developed at the same site of a previously resected dermatofibrosarcoma protuberans. The biopsy specimen from the recurrent tumor revealed malignant fibrous histiocytoma. Immunohistochemistry with anti-CD34 antibody revealed diffuse linear positivity on the tumor cells of dermatofibrosarcoma protuberans and negativity on malignant fibrous histiocytoma. Anti CD34 antibody was valuable for a differential diagnosis of these two lesions.

Original Articles

Extrapleural Solitary Fibrous Tumor A clinical & pathological study of 8 cases.: Mi Kyung Lee, Dong Hwan Shin, Min Sun Cho, Yuon Mee Kim, Jin Kim; Korean J Pathol. 1999;33(2):108-114.

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Abstract: We reviewed eight solitary fibrous tumors occurring at sites other than pleura (three orbit, two retroperitoneum, one each hard palate, thyroid, and tongue) which shared the histologic and immunohistochemical features of solitary fibrous tumors of pleura. Six patients were women, and two were men, aged from 26 to 74 years. The tumors ranging from 1.5 to 19 cm in diameter presented as well-circumscribed, unencapsulated, soft to rubbery tissue masses. Histologically they were characterized by a proliferation of spindle or ovoid cells intervened by a dense bundles of collagen. A variety of growth patterns was identified but the so-called patternless pattern was the predominant one. One tumor exhibited highly cellular sarcomatous areas with extensive necrosis, which was diagnosed as malignant solitary fibrous tumor. Immunohistochemical studies showed that all of the tumors were strongly positive for both CD34 and vimentin, but negative for cytokeratin, S-100 protein, EMA, and desmin. One case examined ultrastructurally showed features of fibroblast. All but one showed no evidence of recurrence or metastasis over follow-up period of 14 to 32 months. We conclude that extrapleural solitary fibrous tumors represent a distinct mesenchymal tumor with variable histologic features and should be differentiated from other spindle cell mesenchymal tumors.

Solitary Fibrous Tumor A clinicopathologic review of five cases.: Bum Kyung Kim, Dong Wook Kang, Kyeong Hee Kim, Seong Ki Min, Jin Man Kim, Kyu Sang Song, Dae Yung Kang, Si Whan Choi; Korean J Pathol. 1999;33(2):115-120.

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Abstract: We experienced five cases of solitary fibrous tumor; two in the pleura, two in the orbital soft tissue, and one in the lung parenchyma. Three patients were male, and the age of the patients ranged from 38 to 71 years (mean age: 53.6). Grossly, the masses were well circumscribed and had varying sizes from 2.5 to 30.0 cm. The cut surfaces were grayish-yellow firm with focal variegated hemorrhage, necrosis, cystic change, and myxoid area. Microscopically, these were characterized by a haphazard proliferation of spindle cells or polygonal cells separated by variable amounts of hyalinized collagen and showed a prominent vascular channels reminiscent of hemangiopericytoma in foci. Immunoperoxidase stains showed a strong reactivity for CD34, and were weakly positive for vimentin. Electron microscopical examination revealed features of fibroblast; spindle to round tumor cells were arranged in groups and surrounded by collagen. Nucleoli were seldom prominent. The cytoplasm contained many microfilaments and a moderate number of cisternae of rough endoplasmic reticulum.

Case Report

Solitary Fibrous Tumor of the Scrotum: A case report.: Jung Weon Shim, Jae Y Ro; Korean J Pathol. 1999;33(4):295-298.

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Abstract PDF: Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that usually arises in the pleura and has been recently reported from unusual locations. We present a case of SFT that occurred in the scrotum. The patient was a 38-year-old man who presented with a painless, left, scrotal mass of five years' duration. Scrotal sonography and pelvic CT scan showed a soft-tissue mass of 11 cm in diameter. The resected tumor measured 11 8 7 cm and was well demarcated from the surrounding soft tissue. The cut surface revealed homogenously grayish-white and gelatinous appearance. No hemorrhage or necrosis was found. Microscopically, the tumor showed hypercellular spindle cell areas intermixed with hypocellular areas lying in a myxoid or collagenous stroma. The spindle cells had no mitosis or low mitotic figures, and little or no nuclear atypia. They exhibited a variety of growth patterns, including "patternless" pattern, and a prominent vasculature with hemangiopericytic pattern. Vimentin, CD34, and bcl-2 protein immunoreactivity were observed. Characteristic histologic and immunohistochemical features of this lesion were consistent with SFT. To arrive at a correct diagnosis of this lesion, especially when it occurs in unusual sites, immunohistochemical study including CD34 & bcl-2 protein is required in addition to characteristic histologic features.

Original Articles

Morphometric Study for Muscular and Microvascular Remodeling of Left Ventricular Free Wall and Interventricular Septum in Hypertrophic Cardiomyopathy.: Young Jik Lee, Hyung Suk Kim, Jong Tae Park, Chang Soo Park; Korean J Pathol. 1999;33(9):675-683.

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Abstract PDF: Hypertrophic cardiomyopathy (HCMP) is characterized myofiber hypertrophy and structural remodeling with changes in the proportion of the muscular, vascular, and interstitial compartments. A study was done to determine the structural remodeling patterns and the role of microvasculature of hearts in HCMP. Forty-two postmortem hearts were analyzed including 14 from patients who died of HCMP (group 1), 8 patients with HCMP but who died from extracardiac causes (group 2), 10 patients with ischemic heart disease (group 3), and 10 normal adult hearts (group 4). Macro- and microscopic examination, immunohistochemical study using CD34 antibody, and morphometric studies using image analyzer were performed. Mean cardiac weight and wall thickness were significantly higher in the HCMP group. Myocardial hypertrophy, and a variety of myocardial disarray and fibrosis involved the whole area of the left ventricles with HCMP. The percentage areas of microvessels were 6.40 0.7 in group 1, 5.90 0.6 in group 2, 4.98 0.3 in group 3, 4.85 0.4 in group 4, respectively, and the numbers of microvessels were 198.0 20.7 in group 1, 230.0 22.3 in group 2, 211.7 11.2 in group 3, and 236.4 11.4 in group 4, respectively (mean SE). The percentage area of microvessels was significantly higher in group 1 than in other groups. However, the number of microvessels in that group was lower than in the other groups, although it was statistically insignificant. Since flow-dependent vasodilation is preserved in HCMP, we considered flow-dependent vasodilation the cause of the discrepancy between the area and the number of microvessels. Ischemic changes observed in chronic HCMP and related heart failure were considesed to be due to the relative deficiency of the coronary flow compared to the increasing cardiac mass.

Tumor Angiogenesis in Renal Cell Carcinoma.: Ji Shin Lee, Jong Jae Jung, Chang Soo Park; Korean J Pathol. 1999;33(11):1055-1060.

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Abstract PDF: Angiogenesis is essential for the growth of solid tumors. Microvessel counts, which represent a measure of tumor angiogenesis, have been correlated with the overall survival of patients with a variety of malignancies. However, the significance of angiogenesis in renal cell carcinoma remains controversial. To determine whether angiogenesis correlates with prognosis of patients with renal cell carcinoma, we counted the microvessels within the primary tumors and compared their numbers with patients' prognosis. Tumor specimens from 42 patients were investigated. Microvessels were stained with anti-CD34 and anti-factor VIII-related antigen monoclonal antibodies. Significant correlation between microvessel counts for two antibodies was observed (r=0.875, p<0.01), although microvessel counts for CD34 were approximately two times higher. Microvessel counts were higher in clear cell than in non-clear cell carcinoma (p<0.05). These results suggest that immunostaining with anti-CD34 antibody may provide a more sensitive and accurate measure of tumor angiogenesis. There was no correlation between microvessel counts and nuclear grade, or TNM stage. In univariate analyses, nuclear grade and TNM stage were significantly associated with patient survival (p<0.01). But further studies on tumor angiogenesis of renal cell carcinoma are needed before it can be adopted as a prognostic marker.

Case Reports

Malignant Gastrointestinal Stromal Tumor of the Esophagus: A Case Report.: Hae Joung Sul, Kyeong Hee Kim, Dae Young Kang; Korean J Pathol. 2001;35(3):252-255.

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Abstract PDF: Gastrointestinal stromal tumors (GISTs) predominate in the stomach and small intestine but have rarely been documented in the esophagus. We report a rare case of GIST of the esophagus in a 47-year-old woman. Histologically, the tumors showed a combination of solid, myxoid, and perivascular collar-like patterns, with spindle and epithelioid cells. The tumor cells were positive for CD117, CD34, and S-100 protein and negative for desmin and -smooth muscle actin.

Dendritic Myxofibrolipoma.: Sung Nam Kim, Kye Hyun Kwon, Yeon Lim Suh; Korean J Pathol. 2001;35(5):447-450.

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Abstract PDF: Dendritic myxofibrolipoma is a recently described disease entity that represents a distinctive benign soft tissue neoplasm showing the combined features of spindle cell lipoma and the solitary fibrous tumor. Immunohistochemical stains reveal a strong positivity for vimentin, CD34 and bcl-2, which highlight the dendritic nature of the tumor cells by demonstrating slender complex cytoplasmic prolongations. There have been 12 cases of dendritic myxofibrolipomas reported in literature. In Korea, none of the cases have been described. We report such a case with a 28-year-old man who had a palpable subcutaneous mass on his right shoulder for 4 months. Grossly, the removed mass measured 11X7X5 cm and appeared to be a well-encapsulated, lipomatous tumor with marked myxoid appearance. Microscopically, this tumor consisted of spindle cells admixed with dense collagen fibers and mature adipocytes in abundant myxoid stroma with high vascularity. Immunohistochemically, the tumor cells were strongly reactive for vimentin and CD34 and weakly reactive for bcl-2, and negative for S-100 protein.

Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts: A Case Report.: Young Chae Chu, Sun Keun Choi, In Suh Park, Hye Seung Han, Jee Young Han, Joon Mee Kim; Korean J Pathol. 2002;36(3):195-198.

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Abstract PDF: A case of rare pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is reported. A 35-year-old woman presented with a subcutaneous solid mass in the left inguinal area, which had been present for 3 months, was presented to us. The tumor was histologically characterized by sheets of mitotically inactive oval and pleomorphic cells, mono-and multinucleated giant cells, intranuclear cytoplasmic inclusions, and prominent clusters of thinwalled ectatic vessels with perivascular hyalinization. A focal hemangiopericytoma-like vascular pattern, pseudovascular spaces, stromal collagen with degenerative change and abundant mast cells were observed. The tumor cells were reactive for vimentin and CD34. This tumor shared several features with malignant fibrous histiocytoma, ancient schwannoma, giant cell angiofibroma, giant cell fibroblastoma and solitary fibrous tumor. The patient was well with no evidence of disease for 10 months.

Gliosarcoma with Components of Anaplastic Oligodendroglioma and Unclassifiable Spindle Cells: A Case Report.: Jung Woo Choi, Youngseok Lee, Jung Suk An, Nam Hee Won, Yong Gu Chung, Yang Seok Chae; Korean J Pathol. 2008;42(1):45-49.

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Abstract PDF: Gliosarcoma is a distinct disease entity that is characterized by a biphasic tissue pattern with alternating areas displaying glial and mesenchymal differentiation. The tumor in our case was a rare morphologic variant of gliosarcoma with components of anaplastic oligodendroglioma and unclassifiable spindle cells. Spindle cells showed CD34 and S-100 protein immunoreactivity, which was possibly related to peripheral nerve sheath differentiation. This unique feature has not been described previously and so this case expands the spectrum of possible divergent mesenchymal differentiation, and it lends support to pluripotential stem cells being the origin of this tumor.

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