Journal of Pathology and Translational Medicine

Case Study

Acquired aberrant partial CD3 expression in recurrent Epstein-Barr virus–negative solitary plasmacytoma of tonsil: Chenchen Niu, Dong Ren, Truc Tran, Ashley Gamayo, Sherif Rezk, Xiaohui Zhao; Received January 5, 2025 Accepted April 17, 2025 Published online May 15, 2025; DOI: https://doi.org/10.4132/jptm.2025.04.17 [Epub ahead of print]

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Abstract PDF: The aberrant expression of specific T-cell maker CD3 in B-cell neoplasms can be a potential diagnostic pitfall leading to a misclassification of cell lineage. Here, we report a case of recurrent solitary plasmacytoma with new aberrant expression of CD3. The neoplastic plasma cells of the recurrent tumor were kappa restricted, positive for CD138, MUM1, negative for CD20, cyclin D1, and Epstein-Barr virus. CD79a was positive in majority of the tumor cells, except for a small focus which was strongly positive for CD3, but negative for other T-cell markers (CD2, CD5, CD7, CD4, and CD8) and CD56. The neoplastic plasma cells of the original tumor were negative for CD3. To the best of our knowledge, only one case of recurrent plasmacytoma with aberrant expression of CD3 has been published, which revealed disease progression in the recurrence. However, we did not observe morphologic evidence of disease progression in our case.

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.

Citations

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Case Study

CD30-Positive T-Cell Lymphoproliferative Disease of the Oral Mucosa in Children: A Manifestation of Epstein-Barr Virus-Associated T-Lymphoproliferative Disorder: Mineui Hong, Young Hyeh Ko; J Pathol Transl Med. 2015;49(6):525-530. Published online September 30, 2015; DOI: https://doi.org/10.4132/jptm.2015.07.13

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Abstract PDF

Eosinophilic ulcer of the oral mucosa (EUOM) is a very rare, benign, self-limiting ulcerative lesion of the oral cavity of unknown pathogenesis, and belongs to the same spectrum of CD30⁺ T-cell lymphoproliferative disease (LPD) of the oral mucosa. The etiology and pathogenesis of the disease are unknown. We report two cases in children who were initially diagnosed with EUOM and CD30⁺ T-cell LPD, respectively. However, retrospective analysis revealed that a majority of infiltrated atypical T cells were positive for Epstein-Barr virus (EBV). The present cases suggest that the pathogenesis and etiology of EUOM or CD30⁺ T-cell LPD occurring in children are different from those in adults. EUOM or CD30⁺ T-cell LPD in children is a manifestation of EBV-positive T-cell LPD, and should therefore be distinguished from the disease in adults.

Citations

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Pediatric oral Epstein-Barr virus associated self-remitting CD30+ lymphoproliferative disorder: A distinct entity
Ziv Schwartz, Robert B. Bowe, Morton Coleman, Cynthia M. Magro
Annals of Diagnostic Pathology.2018; 37: 57. CrossRef

Original Article

ERG Immunohistochemistry as an Endothelial Marker for Assessing Lymphovascular Invasion: Sehun Kim, Hyung Kyu Park, Ho Young Jung, So-Young Lee, Kyueng-Whan Min, Wook Youn Kim, Hye Seung Han, Wan Seop Kim, Tae Sook Hwang, So Dug Lim; Korean J Pathol. 2013;47(4):355-364. Published online August 26, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.4.355

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Abstract PDF

Background

ERG, a member of the ETS family of transcription factors, is a highly specific endothelial marker. We investigated whether the use of ERG immunostaining can help pathologists detect lymphovascular invasion (LVI) and decrease interobserver variability in LVI diagnosis.

Methods

Fifteen cases of surgically resected colorectal cancers with hepatic metastasis were selected and the most representative sections for LVI detection were immunostained with ERG, CD31, and D2-40. Eight pathologists independently evaluated LVI status on hematoxylin and eosin (H&E) and the corresponding immunostained sections and then convened for a consensus meeting. The results were analyzed by kappa (κ) statistics.

Results

The average rate of LVI positivity was observed in 43% with H&E only, 10% with CD31, 29% with D2-40, and 16% with ERG. Agreement among pathologists was fair for H&E only (κ=0.27), D2-40 (κ=0.21), ERG (κ=0.23), and was moderate for CD31 (κ=0.55). Consensus revealed that ERG nuclear immunoreactivity showed better visual contrast of LVI detection than the other staining, with improved agreement and LVI detection rate (κ=0.65, LVI positivity rate 80%).

Conclusions

The present study demonstrated a superiority with ERG immunostaining and indicated that ERG is a promising panendothelial marker that might help pathologists increase LVI detection and decrease interobserver variability in LVI diagnosis.

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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.

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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

Sclerosing Angiomatoid Nodular Transformation (SANT) in Spleen: A Case Report.: Hyun Jung Lee, Song Yi Choi, Song Mei Huang, Ji Young Sul, Jin Man Kim; Korean J Pathol. 2011;45(1):111-114.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.1.111

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Abstract PDF

Sclerosing angiomatoid nodular transformation (SANT) of spleen is a rare inflammatory tumor-like vascular lesion composed of angiomatoid nodules in a fibrosclerotic background. We report herein on a case of SANT in the spleen with its pathologic features, and review the related literature. A 50-year-old woman presented with mild left upper quadrant discomfort and tenderness and she showed a 6 cm-sized solitary splenic mass on computed tomography. She underwent laparoscopic splenectomy. Grossly, the spleen showed a well circumscribed round-shaped solid mass with multinodular hemorrhagic surfaces. Microscopically, the mass consisted of multiple angiomatoid nodules surrounded by collagen bundles with fibroblasts and a lymphoplasma cell infiltration. Immunohistochemically, the cells of the angiomatoid nodules were positive for CD31, CD30, CD34, alpha-smooth muscle actin, and VWF-VIII, but they were negative for CD8, anaplastic lymphoma kinase protein, and D2-40. The patient has been under close follow-up without recurrence.

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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.

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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

Clinico-Pathologic Evaluation of 18 Cases of Lymphomatoid Papulosis.: Sug Kyoung Ko, Hye Sook Kim, Kee Suck Suh, Sang Tae Kim, Man Ha Huh; Korean J Pathol. 1996;30(6):505-514.

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Abstract PDF: Lymphomatoid papulosis is an enigmatic disease entity which is clinically benign and histologically malignant. Although sporadic cases have been reported, we could not find any comprehensive report on the combined clinical and histologic features of lymphomatoid papulosis in the literature. Perhaps the most controversial aspect of lymphomatoid papulosis is its pathogenesis and categorization as a benign versus a malignant entity. To date, there are no reports on p53 and bcl-2 protein expression in lymphomatoid papulosis. We analysed the clinico-pathological findings of 18 cases with lymphomatoid papulosis during the 10 year period from 1984 to 1995 and examined the prevalence of immunoreactivity for CD30(DAKO, Ber-H2), p53(DAKO, DO-7), and bcl-2(DAKO, 124) using an immunohistochemical(ABC) method. The results obtained are summarized as follows. 1) Age distribution ranged from 20 to 65, with a mean age of 45 years and a sex distribution which showed a male predominence(8:1). The lesions were located on the trunk and extremities(8cases), extremities (7cases), and trunk(3 cases). The morphology of the lesions were papules or plaques(12 cases), and nodules(6 cases). 2) Histopathologic types were classified into 3 types: type A(4 cases), type B(8 cases) and mixed type (6 cases). 3) Positive immunoreactivity for CD30 was seen in 17%(3 of 18cases): type A(2 of 3) and mixed type(1 of 3). 4) The positive immunoreactivity for p53 and bcl-2 was observed in 29%(5 of 18) and 11%(2 of 18), respectively. 5) Cases showing positive immunoreactivity for P53 were type A(1 of 5), type B(1 of 5), and mixed type(3 of 5). 6) Cases showing positive immunoreactivity for bcl-2 were mixed type(2 of 2). One case developed into Ki-1 lymphoma. These results support the idea that lymphomatoid papulosis and Ki-1 lymphoma represent a continuum. The role of p53 gene mutation and bcl-2 activation in the development of lymphomatoid papulosis is currently unknown. But, our results suggest that p53 gene mutation and bcl-2 activation are not a critical step in the development of lymphomatoid papulosis. Further studies are needed to elucidate the role of p53 gene mutation and bcl-2 activation in the development and progression of lymphomatoid papulosis.

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.

Correlation between Tumor Angiogenesis (Microvessel Density), Metastasis and Tumor Cell Proliferation in Colorectal Carcinomas.: Young Chae Chu, Joon Mee Kim; Korean J Pathol. 1997;31(6):517-526.

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Abstract PDF: Tumor angiogenesis has been shown to be associated with metastatic potentials in breast, lung and prostatic carcinomas. The relation between tumor angiogenesis and metastatic potentials in colorectal cancer has not been established to date. We analysed 66 selected patients with colorectal carcinomas (37 with and 29 without nodal metastases) for the microvessel density, tumor proliferation activity, and the clinicopathologic parameters including size, stage, histologic grade, growth pattern, presence of angioinvasion, perineural invasion and lymph node metastasis. For evaluation of microvessel density and tumor proliferative activity, the primary tumors were immunohistochemically stained for CD31 and PCNA. The mean microvessel counts (MVC) per 200X field were 99.27+/-23.28 and 131.35+/-31.48 in node-negative and node-positive patients, respectively. The PCNA index was 39.41+/-5.63% and 56.60+/-7.09% in node-negative and node-positive patients, respectively. MVC and PCNA index were higher in tumors with nodal metastasis (p=0.002, p<0.001), and also correlated each other (sr=0.33, p=0.007). Higher microvessel counts were seen in tumors with advanced stage (p=0.016). Tumor proliferation activity assessed by PCNA immunostaining was significantly higher in tumors with advanced stage, perineural invasion, angioinvasion, poor differentiation and larger size. From these results, MVC and PCNA index in colorectal carcinomas are assumed to be valuable prognostic parameters. Thus assessment of tumor angiogenesis and tumor cell proliferation in colorectal carcinomas may be helpful for the patients in need of aggressive therapy.

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.

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