Journal of Pathology and Translational Medicine

Case Studies

Primary epithelioid inflammatory myofibroblastic sarcoma of the brain with EML4::ALK fusion mimicking intra-axial glioma: a case report and brief literature review: Eric Eunshik Kim, Chul-Kee Park, Koung Mi Kang, Yoonjin Kwak, Sung-Hye Park, Jae-Kyung Won; J Pathol Transl Med. 2024;58(3):141-145. Published online May 14, 2024; DOI: https://doi.org/10.4132/jptm.2024.04.12

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Abstract PDF: An aggressive subtype of inflammatory myofibroblastic tumor, epithelioid inflammatory myofibroblastic sarcoma occurs primarily inside the abdominal cavity, followed by a pulmonary localization. Most harbor anaplastic lymphoma kinase (ALK) gene rearrangements, with RANBP2 and RRBP1 among the well-documented fusion partners. We report the second case of primary epithelioid inflammatory myofibroblastic sarcoma of the brain, with a well-known EML4::ALK fusion. The case is notable for its intra-axial presentation that clinico-radiologically mimicked glioma.

Primary pulmonary epithelioid inflammatory myofibroblastic sarcoma: a rare entity and a literature review: Priyanka Singh, Aruna Nambirajan, Manish Kumar Gaur, Rahul Raj, Sunil Kumar, Prabhat Singh Malik, Deepali Jain; J Pathol Transl Med. 2022;56(4):231-237. Published online July 7, 2022; DOI: https://doi.org/10.4132/jptm.2022.05.08

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Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of inflammatory myofibroblastic tumor (IMT) harboring anaplastic lymphoma kinase (ALK) gene fusions and is associated with high risk of local recurrence and poor prognosis. Herein, we present a young, non-smoking male who presented with complaints of cough and dyspnoea and was found to harbor a large right lower lobe lung mass. Biopsy showed a high-grade epithelioid to rhabdoid tumor with ALK and desmin protein expression. The patient initially received 5 cycles of crizotinib and remained stable for 1 year; however, he then developed multiple bony metastases, for which complete surgical resection was performed. Histopathology confirmed the diagnosis of EIMS, with ALK gene rearrangement demonstrated by fluorescence in situ hybridization. Postoperatively, the patient is asymptomatic with stable metastatic disease on crizotinib and has been started on palliative radiotherapy. EIMS is a very rare subtype of IMT that needs to be included in the differential diagnosis of ALKexpressing lung malignancies in young adults.

Citations

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Mediastinal epithelioid inflammatory myofibroblastic sarcoma with the EML4‐ALK fusion: A case report and literature review
Tingyu Pan, Xinyu Sun, Xiao Wu, Futing Tang, Xianmei Zhou, Qian Wang, Shi Chen
Respirology Case Reports.2024;[Epub] CrossRef
Primary epithelioid inflammatory myofibroblastic sarcoma of the brain with EML4::ALK fusion mimicking intra-axial glioma: a case report and brief literature review
Eric Eunshik Kim, Chul-Kee Park, Koung Mi Kang, Yoonjin Kwak, Sung-Hye Park, Jae-Kyung Won
Journal of Pathology and Translational Medicine.2024; 58(3): 141. CrossRef
Epithelioid Inflammatory Myofibroblastic Sarcoma: A Report of a Rare Case
Varun Ronanki, Vaddatti Tejeswini, Inuganti Venkata Renuka, Shaik Raheema, Bakkamanthala S K Kanth
Cureus.2024;[Epub] CrossRef
Thoracic epithelioid inflammatory myofibroblastic sarcoma: a rare and aggressive disease with case report and literature review
Linke Yang, Pei Li, Runze Liu, Baomin Feng, Huiqing Mao, Xiaoyong Tang, Guangjian Yang
Discover Oncology.2024;[Epub] CrossRef
Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib
Mengmeng Li, Ruyue Xing, Jiuyan Huang, Chao Shi, Chunhua Wei, Huijuan Wang
Frontiers in Oncology.2023;[Epub] CrossRef
Epithelioid Inflammatory Myofibroblastic Sarcoma With Poor Response to Crizotinib: A Case Report
Soheila Aminimoghaddam, Roghayeh Pourali
Clinical Medicine Insights: Case Reports.2023;[Epub] CrossRef
Epithelioid inflammatory myofibroblastic sarcoma: a case report and brief literature review
Weidong Dou, Yu Guan, Tao Liu, Hang Zheng, Shuo Feng, Yingchao Wu, Xin Wang, Zhanbing Liu
Frontiers in Oncology.2023;[Epub] CrossRef

Original Article

An Immunohistochemical and Polarizing Microscopic Study of the Tumor Microenvironment in Varying Grades of Oral Squamous Cell Carcinoma: Aeman Khalid, Safia Siddiqui, Bharadwaj Bordoloi, Nafis Faizi, Fahad Samadi, Noora Saeed; J Pathol Transl Med. 2018;52(5):314-322. Published online July 27, 2018; DOI: https://doi.org/10.4132/jptm.2018.07.17

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Abstract PDF

Background
Invasion of epithelial cells into the connective tissue brings about massive morphological and architectural changes in the underlying stroma. Myofibroblasts reorganize the stroma to facilitate the movement of tumor cells leading to metastasis. The aim of this study was to determine the number and pattern of distribution of myofibroblasts and the qualitative and quantitative change that they cause in the collagen present in the stroma in various grades of oral squamous cell carcinoma (OSCC).
Methods
The study was divided into two groups with group I (test group, 65 cases) consisting of 29 cases of well-differentiated squamous cell carcinoma, 25 moderately differentiated SCC, and 11 poorly differentiated SCC, and group II (control group) consisting of 11 cases of normal mucosa. Sections from each sample were stained with anti–α-smooth muscle actin (α-SMA) antibodies, hematoxylin and eosin, and Picrosirius red. Several additional sections from each grade of OSCC were stained with Masson’s trichrome to observe the changes in collagen. For the statistical analysis, Fisher’s exact test, Tukey’s post hoc honest significant difference test, ANOVA, and the chi-square test were used, and p < .05 was considered statistically significant.
Results
As the tumor stage progressed, an increase in the intensity α-SMA expression was seen, and the network pattern dominated in more dedifferentiated carcinomas. The collagen fibers became thin, loosely packed, and haphazardly aligned with progressing cancer. Additionally, the mean area fraction decreased, and the fibers attained a greenish yellow hue and a weak birefringence when observed using polarizing light microscopy.
Conclusions
Myofibroblasts bring about numerous changes in collagen. As cancer progresses, there isincrease in pathological collagen,which enhances the movement of cells within the stroma.

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Multifractal Alterations in Oral Sub-Epithelial Connective Tissue During Progression of Pre-Cancer and Cancer
Debaleena Nawn, Sawon Pratiher, Subhankar Chattoraj, Debjani Chakraborty, Mousumi Pal, Ranjan Rashmi Paul, Srimonti Dutta, Jyotirmoy Chatterjee
IEEE Journal of Biomedical and Health Informatics.2021; 25(1): 152. CrossRef

Case Study

Mammary-Type Myofibroblastoma: A Report of Two Cases: Soyeon An, Joon Seon Song, Soonchan Park, Jung Won Lee, Kyung-Ja Cho; J Pathol Transl Med. 2016;50(5):385-389. Published online June 6, 2016; DOI: https://doi.org/10.4132/jptm.2016.03.26

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Abstract PDF

Mammary-type myofibroblastoma (MFB) is a rare, benign spindle cell neoplasm occurring along the milkline, with extension from the mid-axilla to the medial groin. It is histologically and immunohistochemically identical to MFB of the breast and is part of a spectrum of lesions that includes spindle cell lipoma and cellular angiofibroma. Recently, we experienced two cases of mammary-type MFB involving male patients aged 30 and 58 years, respectively. The tumors were located in the right scrotal sac and in the right axilla. Wide excisions were performed. Microscopically, the masses were composed of haphazardly arranged, variably sized fascicles of bland spindle cells and were admixed with mature fat tissue. The spindle cells in both cases showed immunopositivity for desmin and CD34 and negativity for smooth muscle actin. Loss of retinoblastoma (RB)/13q14 loci is a characteristic genetic alteration of mammary-type MFB, and we identified loss of RB protein expression by immunohistochemical staining. We emphasize the importance of awareness of this rare neoplasm when a spindle cell neoplasm is accompanied by desmin immunopositivity. The second patient was alive without recurrence for 20 months, and the first patient had not been followed.

Citations

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Myofibroblastoma in the Liver: A Case Report and Review of Literature
Mohan Narasimhamurthy, Deepika Savant, Lauren Shreve, Mark A. Rosen, Major Kenneth Lee, Kumarasen Cooper, Emma E. Furth, Paul J. Zhang, Zhaohai Yang
International Journal of Surgical Pathology.2023; 31(8): 1559. CrossRef
Mammary-type myofibroblastoma of the thigh mimicking liposarcoma
Natasha Akhlaq, Bibianna Purgina, Joel Werier, Zaid Jibri
Skeletal Radiology.2022; 51(2): 441. CrossRef
Mammary‐type myofibroblastoma of the perineum: Typical or rare location?
Akihiro Naito, Yuta Takeshima, Sayuri Takahashi
IJU Case Reports.2022; 5(3): 161. CrossRef
Identifying the uncommon solitary fibrous tumour in a rare location – A case report
Shanthi Periasamy, Anita Mani, Graham J. Stewart, Jacob P. Hampton
International Journal of Surgery Case Reports.2022; 94: 107058. CrossRef
Mammary-Type Myofibroblastoma of Perineal Region: A Case Report and Literature Review
晓虹江
Advances in Clinical Medicine.2021; 11(04): 1722. CrossRef
A case of mammary-type myofibroblastoma of the inguinal region
Atsushi Ishihara, Takeo Yasuda, Yukari Sakae, Masayuki Sakae, Tooru Hamada, Hideki Tsukazaki, Takashi Tsukazaki, Masaru Furumoto
International Journal of Surgery Case Reports.2018; 53: 464. CrossRef
Radiologic presentation of a myofibroblastoma of the adult male breast
Evan Rochlis, Pauline Germaine
Radiology Case Reports.2017; 12(3): 439. CrossRef
Imaging features of mammary-type myofibroblastoma of soft tissue: a case series with literature review
Gokhan Kuyumcu, Brian P. Rubin, Carl Winalski
Skeletal Radiology.2017; 46(9): 1283. CrossRef
Mammary-type myofibroblastoma of the psoas
Kamran Khan, Arun A Mavanur
BMJ Case Reports.2016; : bcr2016217862. CrossRef

Original Article

Endogenous Gastric-Resident Mesenchymal Stem Cells Contribute to Formation of Cancer Stroma and Progression of Gastric Cancer: Eun-Kyung Kim, Hye-Jung Kim, Young-Il Yang, Jong Tae Kim, Min-Young Choi, Chang Soo Choi, Kwang-Hee Kim, Jeong-Han Lee, Won-Hee Jang, Soon-Ho Cheong; Korean J Pathol. 2013;47(6):507-518. Published online December 24, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.6.507

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Abstract PDF

Background

Carcinoma-associated fibroblasts (CAFs) contribute to carcinogenesis and cancer progression, although their origin and role remain unclear. We recently identified and investigated the in situ identity and implications of gastric submucosa-resident mesenchymal stem cells (GS-MSCs) in the progression of gastric carcinogenesis.

Methods

We isolated GS-MSCs from gastric submucosa using hydrogel-supported organ culture and defined their identity. Isolated cells were assessed in vitro by immunophenotype and mesengenic multipotency. Reciprocal interactions between GS-MSCs and gastric cancer cells were evaluated. To determine the role of GS-MSCs, xenografts were constructed of gastric cancer cells admixed with or without GS-MSCs.

Results

Isolated cells fulfilled MSCs requirements in regard to plastic adherence, stromal cell immunophenotype, and multipotency. We demonstrated a paracrine loop that gastric cancer cells enhanced the migration, proliferation, and differentiation of GS-MSCs; additionally, GS-MSCs promoted the proliferation of gastric cancer cell in vitro. Xenograft experiments showed that GS-MSCs significantly promoted cancer growth and angiogenesis. GS-MSCs that integrated into gastric cancer became not only CAFs but also rarely endothelial cells which contributed to the formation of cellular and vascular cancer stroma.

Conclusions

Endogenous GS-MSCs play an important role in gastric cancer progression.

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Peiyuan Li, Huan Zhang, Tao Chen, Yajing Zhou, Jiaoyang Yang, Jin Zhou
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Case Study

Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review: Yuil Kim, Ha Young Park, Junhun Cho, Joungho Han, Eun Yoon Cho; Korean J Pathol. 2013;47(2):172-176. Published online April 24, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.2.172

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Abstract PDF

Congenital peribronchial myofibroblastic tumor (CPMT) is a benign pulmonary spindle cell neoplasm of intrauterine and perinatal period, which is thought to arise from primitive peribronchial mesenchyme. We present a case detected incidentally in a one-month-old infant. The solid and partially necrotic tumor involved the right middle and lower lobes of the lung with extension to the diaphragm. Histologically, the tumor was composed of fasciculated monotonous spindle cells, proliferating peribronchiolar cartilage and round cells with rich vasculature, and high mitotic activity was identified in the round cell area. Immunohistochemical and electron microscopic studies showed that the spindle cells were myofibroblastic in phenotype. Although the tumor showed several malignant pathological features, recurrence was not observed in the two-year follow-up period, consistent with the benign clinical behavior of CPMT.

Citations

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Congenital peribronchial myofibroblastic tumor (CPMT): a case report with long term follow-up and next-generation sequencing (NGS)
Ping Zhou, Shuang Li, Weiya Wang, Yuan Tang, Lili Jiang
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Prenatal imaging and immunohistochemical analysis of congenital peribronchial myofibroblastic tumor
Y.‐A. Tu, W.‐C. Lin, H.‐J. Chen, J.‐C. Shih
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A Congenital Peribronchial Myofibroblastic Tumor Detected in a Premature Infant at 28 Weeks but That Resolved in the Late Stage of Pregnancy
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Congenital peribronchial myofibroblastic tumor
Yuka Hotokebuchi, Kenichi Kohashi, Satoshi Toyoshima, Naoko Matsumoto, Toshinori Nakashima, Yoshinao Oda
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Case Report

Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: Report of Two Cases and Review of the Literature: Youngran Kang, Wonkyung Jung, In-Gu Do, Eui Jin Lee, Min Hyeong Lee, Kyoung-Mee Kim, Jongsang Choi; Korean J Pathol. 2012;46(3):292-296. Published online June 22, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.3.292

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Abstract PDF

Plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach is a recently recognized entity. Because of its rarity, only 22 cases have been reported in the English-language literature and most of these are single case reports. We report two cases of gastric PAMT. The tumor cells were bland and plexiform arranged in a myxoid stroma, which was positive for alcian blue. Immunohistochemically, the tumor cells were positive for smooth muscle actin, but negative for c-kit, CD34, desmin, S-100 protein, epithelial membrane antigen, neurofilament, and protein kinase C-theta. Mutation analyses for exon 9, 11, 13, and 17 of KIT genes and 12, 14, and 18 of the platelet-derived growth factor receptor alpha (PDGFRA) genes were performed and the tumors were wild-type for mutation.

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Plexiform Fibromyxoma in the Stomach: Immunohistochemical Profile and Comprehensive Genetic Characterization
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Pediatric and Developmental Pathology.2014; 17(1): 21. CrossRef

Short Case Report

Intranodal Palisaded Myofibroblastoma with Desmin Expression : A Brief Case Report.: Dong Chul Kim, Tae Hoon Kang, Min A Kim, Yoon Kyung Jeon; Korean J Pathol. 2009;43(3):263-265.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.263

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Abstract PDF: Intranodal palisaded myofibroblastoma is a rare benign mesenchymal neoplasm of the lymph node. It is characterized by intranodal spindle cell proliferation along with amianthoid fibers and prominent hemorrhage. It has been rarely reported in South Korea. We report here on a case of palisaded myofibroblastoma that arose in the left inguinal lymph node. The tumor mass was well demarcated, and it was composed of a proliferation of benign-looking spindle cells. It showed focal hemorrhage and a fibrous pseudocapsule. The tumor cells displayed little pleomorphism, no mitotic count, and characteristic palisading nuclei and amianthoid fibers. The tumor cells were positive for smooth muscle actin, vimentin, and also for desmin, but they were negative for S-100 protein, supporting the diagnosis of myofibroblastoma.

Case Report

Angiomyofibroblastoma of Vulva: A case report.: Hye Kyung Lee, Myung Jin Joo, Kwang Min Lee, Dong Kyu Chung; Korean J Pathol. 1995;29(4):530-532.

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Abstract PDF: Angiomyofibroblastoma of the vulva is a rare mesenchymal tumor. It has been diagnostically confused with aggressive angiomyxoma which has a somewhat different clinical course and therapy. Herein we report a case of angiomyofibroblastoma of the vulva in a 46-year-old female. Microscopically, there were alternating hypercellular and hypocelluar edematous zones in which abundant capillary blood vessels were irregularly distributed. Spindle, plump spindle, and oval stromal cells were concentrated around the blood vessels, or loosely dispersed in the hypocellular area. Immunohistochemically, the stromal cells were positive for desmin, vimentin, muscle-specific actin and weakly positive for S-100 protein. Ultrastructural studies showed well developed rough endoplasmic reticulum, abundant intermediate filaments, and pinocytic vesicles in the stromal cells.

Original Article

Immunohistochemical and Ultrastructural Study of Fibroblast Differentiation.: Chae Hong Suh; Korean J Pathol. 1996;30(2):106-114.

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Abstract PDF: The histogenesis of the myofibroblast continues to be a controversial issue. The most popular view is that the myofibroblast is derived directly from the fibroblast. The important role of myofibroblasts in the synthesis of collagen and in wound contraction was demonstrated initially in granulation tissue in experimental animals. Four settings are recognized in which myofibroblasts are the principal proliferative cells: reparative responses, pseudoneoplastic disorders, stromal response to neoplasia, and true neoplasms, both benign and malignant. To identify of fibroblastic cells with smooth muscle differentiation features in the nonneoplastic and neoplastic lesions, we examined a variety of histological, immunohistochemical and ultrastructural features of 7 cases of granulation tissue, 7 of hypertrophic scar, 10 of chronic persistent hepatitis, 10 of chronic active hepatitis, 7 of liver cirrhosis, 7 of fibromatosis, 42 of cervical intraepithelial neoplasia, 14 of microinvasive carcinoma, 14 of invasive carcinoma, 7 of fibroma, 20 of fibrosarcoma and 72 of malignant fibrous histiocytoma. Antibodies against alpha-smooth muscle actin and desmin were used in a biotin-streptavidin procedures. The results of immunohistochemical and electron microscopical examinations yielded virtually identical findings. The identification of fibroblastic cells with smooth muscle cell differentiation features in the desmoplastic reactions of carcinomas, fibroma, fibrosarcoma and malignant fibrous histiocytoma offers also novel diagnostic and prognostic perspectives, that might help in evaluating preneoplastic lesions and malignant lesions. So degree of proliferative myofibroblasts was helpful diagnostic aid in differentiation of chronic persistent hepatitis, chronic active hepatitis and liver cirrhosis.

Case Report

Angiomyofibroblastoma of the Vulva: A case report.: Mee Sook Roh, Hea Kyoung Hur, Sook Hee Hong, Sang Kap Kim, Young Cheol Baek, Hwa Sook Moon; Korean J Pathol. 1996;30(4):344-346.

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Abstract PDF: Angiomyofibroblastoma is a distinctive, hitherto uncharacterized, benign soft tissue tumor of the vulva with histology similar to an aggressive pelvic angiomyxoma. It can be distinguished from an aggressive angiomyxoma by its circumscribed borders, higher cellularity, more numerous blood vessels, the frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation. We experienced a case of angiomyofibroblastoma of the vulva occurring in a 45-year-old woman. The lesion was a well-defined but not encapsulated mass, 4.5x4.2 cm. Histologically the mass was characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels were irregularly distributed. Immunohistochemically, the spindled, plump spindled, and oval stromal cells were reactive for vimentin and desmin, but not for cytokeratin, or S-100 protein.

Original Article

Inflammatory Pseudotumor of the Urinary Bladder: An Immunohistochemical and Ultrastructural Study.: Seung Sam Paik, Joo Seob Keum, Moon Hyang Park, Jung Dal Park; Korean J Pathol. 1996;30(5):447-452.

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Abstract PDF: Inflammatory pseudotumor of the urinary bladder is an unusual, benign mesenchymal proliferative lesion of the submucosal stroma easily mistaken for a malignant neoplasm clinically and histologically. We present a case and describe the clinical presentation and radiologic, histologic, histochemical, immunohistochemical, and ultrastructural findings. A 23-year old patient presented with sudden onset of gross painless hematuria for 3 months. There was no previous instrumentation or surgery involving the genitourinary tract. Cystoscopy revealed a large polypoid and ulcerated bladder mass. The lesion consisted of plump spindle shaped, fibroblast-like cells embedded in a myxoid stroma. Mitotic figures were negligible and the lesion showed encroachment on the superficial muscle bundles. The spindle cells were immunoreactive for vimentin and muscle specific actin. Immunohistochemical and ultrastructural findings revealed the fibroblastic-myofibroblastic nature of this lesion. Complete surgical excision by partial cystectomy was successful in eradicating the lesion. The findings are described with a discussion of the pathogenesis and review of the literature.

Case Reports

Myofibroblastoma of the Male Breast: Report of a case.: Ji Eun Kim, Yeon Lim Suh, Howe Jung Ree; Korean J Pathol. 1996;30(7):623-629.

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Abstract: A case of myofibroblastoma of the breast in a 55-year-old man is described. Myofibroblastoma is a relatively recently recognized benign stromal tumor, and predominantly occurs in middle aged men. The pateint presented with a nontender firm mass in his right breast. Fine needle aspiration biopsy revealed bland looking stromal cell clusters without epithelial cells. Simple excision was done and the patient discharged uneventfully. The mass was well demarcated, lobulated and sligtly myxoid. Microscopically bipolar elongated spindle cell fascicles with interspersing broad collagen bands are so characteristic. Ultrastructurally the tumor cell show features of fibroblast as well as smooth muscle cell.

Recurred Angiomyofibroblastoma of the Vulva: Report of a case.: Do Youn Park, Ji Yeon Kim, OK Hyeon Kim, Hwa Sun Lee, Mee Young Sol, Kang Suek Suh, Sun Kyung Lee; Korean J Pathol. 1996;30(10):947-950.

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Abstract PDF: Angiomyofibroblastoma is a rare, benign mesenchymal tumor of the vulva. Since it was described in 1992 by Fletcher, 15 cases have been reported in literature. We recently experienced a recurred angiomyofibroblastoma of the vulva. A 45-year-old woman was presented initially in 1991 with a mass of labium major and local excision of tumor mass had been performed. A histologic diagnosis was made of angiomyxoma, but this diagnosis was revised to angiomyofibroblastoma by the authors. The recurred mass was well circumscribed, measuring 2.5x1.6x1.5cm in dimensions. Microscopically the tumor was characterized by high cellularity, numerous blood vessels(which lack prominent hyalinization), and plump stromal cells. Immunohistochemically, the stromal cells were reactive for vimentin and desmin, but not alpha-smooth muscle actin, or S-100 protein. We thought that this case was a recurred angiomyofibrblastoma of the vulva due to incomplete surgical excision.

Inflammatory Myofibroblastic Tumor in Posterior Mediastinum.: Seung Sam Paik, Seok Hoon Jeon, Se Jin Jang, Moon Hyang Park, Jung Dal Lee; Korean J Pathol. 1997;31(1):63-67.

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Abstract PDF: Inflammatory myofibroblastic tumor(IMT) or inflammatory pseudotumor is a rare, solid tumor that most often affects children. This tumor is characterized by a spindle cell proliferation admixed with a variety of inflammatory cells. Although it has disputed nosology, a distinctive fibroinflammatory and even pseudosarcomatous appearance have been well appreciated. Herein, we report a case of IMT in the posterior mediastinum in a 19-year-old girl with clinical findings. The immunohistochemical and ultrastructural studies on the tumor cells are reported, and their distinctive characteristics are discussed in details.

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