Journal of Pathology and Translational Medicine

Case Studies

A sinonasal yolk sac tumor in an adult: Jaehoon Shin, Ji Heui Kim, Kyeong Cheon Jung, Kyung-Ja Cho; J Pathol Transl Med. 2022;56(3):152-156. Published online January 26, 2022; DOI: https://doi.org/10.4132/jptm.2021.12.09

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Yolk sac tumors (YSTs), which are also called endodermal sinus tumors, are malignant tumors of germ cell origin. These tumors usually occur in the gonads, but 20% of cases have been reported at extragonadal sites. The head and neck is a rarely affected region that accounts for just 1% of all malignant tumors of germ cell origin. In addition, YSTs arise mostly in childhood. We present a rare pathologically pure case of primary adult YST in the sinonasal area. A 45-year-old male patient presented with a rapidly growing mass in the nasal cavity, which caused nasal obstruction and bloody post-nasal drip. The histopathologic features indicated pure YST, and immunohistochemical analysis revealed positive reactivity for Sal-like protein 4 and alpha-fetoprotein. Herein, we discuss the clinical, radiologic, and histologic features of this YST and review other cases of sinonasal YST in adults.

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International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors
Edward C. Kuan, Eric W. Wang, Nithin D. Adappa, Daniel M. Beswick, Nyall R. London, Shirley Y. Su, Marilene B. Wang, Waleed M. Abuzeid, Borislav Alexiev, Jeremiah A. Alt, Paolo Antognoni, Michelle Alonso‐Basanta, Pete S. Batra, Mihir Bhayani, Diana Bell,
International Forum of Allergy & Rhinology.2024; 14(2): 149. CrossRef
Yolk sac tumor of postpubertal-type does not exhibit immunohistochemical loss of SMARCB1/INI1 and SMARCA4/BRG1…but choriocarcinoma?
Costantino Ricci, Francesca Ambrosi, Tania Franceschini, Francesca Giunchi, Eugenia Franchini, Francesco Massari, Veronica Mollica, Federico Mineo Bianchi, Maurizio Colecchia, Andres Martin Acosta, Michelangelo Fiorentino
Pathology - Research and Practice.2023; 241: 154269. CrossRef
Pure yolk sac tumor primarily in the nasal cavity: A case report
Zijun Liu, Baohong Wen, Yan Zhang
Asian Journal of Surgery.2023; 46(10): 4712. CrossRef
A case of Yolk sac tumor arising from paranasal sinus
Kaori Shinomura, Munehito Moriyama, Keigo Fujita, Takashi Hirano, Masashi Suzuki
JOURNAL OF JAPAN SOCIETY FOR HEAD AND NECK SURGERY.2023; 33(1): 41. CrossRef
A Novel Successful Case of Nasal and Sinus Yolk Sac Tumor With SMARCB1 (INI-1) Deficiency: A Case Report
Tianyu He, Zhiyu Wang, Hongbo Su, Sihan Li, Zheng He
Cureus.2022;[Epub] CrossRef

Primary hepatic mixed germ cell tumor in an adult: Hyun-Jung Sung, Jihun Kim, Kyu-rae Kim, Shinkyo Yoon, Jae Hoon Lee, Hyo Jeong Kang; J Pathol Transl Med. 2021;55(5):355-359. Published online August 3, 2021; DOI: https://doi.org/10.4132/jptm.2021.06.16

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Abstract PDF

Primary hepatic mixed germ cell tumor (GCT) is very rare, and less than 10 cases have been reported. We report a case of mixed GCT composed of a choriocarcinoma and yolk sac tumor, which occurred in the liver of a 40-year-old woman. A large mass was detected by computed tomography solely in the liver. Serum β-human chorionic gonadotropin (hCG) was highly elevated, otherwise, other serum tumor markers were slightly elevated or within normal limits. For hepatic choriocarcinoma, neoadjuvant chemotherapy was administered, followed by right lobectomy. Histologic features of the resected tumor revealed characteristic choriocarcinoma features with diffuse positivity for hCG in the syncytiotrophoblasts and diffuse positivity for α-fetoprotein and Sal-like protein 4 in the yolk sac tumor components. Primary malignant GCT in the liver is associated with a poor prognosis and requires specific treatment. Therefore, GCT should be considered during a differential diagnosis of a rapidly growing mass in the liver.

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Testicular Seminoma in Prostate: Case Report and Review of Literature
Peter Lesko, Jana Obertova, Karol Kajo, Katarina Rejlekova, Zuzana Orszaghova, Viera Lehotska, Martina Ondrusova, Michal Chovanec, Dalibor Ondrus, Michal Mego
Clinical Genitourinary Cancer.2024; 22(2): 210. CrossRef

Malignant rhabdoid tumor of the kidney in an adult with loss of INI1 expression and mutation in the SMARCB1 gene: Eunkyung Han, Jiyoon Kim, Min Jung Jung, Susie Chin, Sang Wook Lee, Ahrim Moon; J Pathol Transl Med. 2021;55(2):145-153. Published online March 9, 2021; DOI: https://doi.org/10.4132/jptm.2021.01.26

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Abstract PDF

A 57-year-old man with left flank pain was referred to our institute. Computed tomography scans revealed two enhancing masses in the left kidney. The clinical diagnosis was renal cell carcinoma (RCC). He underwent a radical nephrectomy with an adrenalectomy. Two well-circumscribed solid masses in the hilum and the lower pole (4.5 × 3.5 cm and 7.0 × 4.1 cm) were present. Poorly cohesive uniform round to polygonal epithelioid cells making solid sheets accounted for most of the tumor area. The initial diagnosis was RCC, undifferentiated with rhabdoid features. As the tumor showed loss of INI1 expression and a mutation in the SMARCB1 gene on chromosome 22, the revised diagnosis was a malignant rhabdoid tumor (MRT) of the kidney. To date, only a few cases of renal MRT in adults have been reported. To the best of our knowledge, this is the first report of MRT in the native kidney of an adult demonstrating a SMARCB1 gene mutation, a hallmark of MRT.

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Supratentorial ATRT in a young Infant: Expanding the diagnostic spectrum beyond medulloblastoma
Ali Msheik, Mohamad Aoun, Youssef Fares
Interdisciplinary Neurosurgery.2024; 35: 101857. CrossRef
Malignant rhabdoid tumor of kidney in an adult patient with positive family history of rhabdoid tumor: A case report and review of literature
Farhood Khaleghi mehr, Nasrollah Abian, Mandana Rahimi, Yasaman Moradi
International Journal of Surgery Case Reports.2023; 113: 109053. CrossRef

Original Article

Comparison of Squamous Cell Carcinoma of the Tongue between Young and Old Patients: Gyuheon Choi, Joon Seon Song, Seung-Ho Choi, Soon Yuhl Nam, Sang Yoon Kim, Jong-Lyel Roh, Bu-Kyu Lee, Kyung-Ja Cho; J Pathol Transl Med. 2019;53(6):369-377. Published online October 11, 2019; DOI: https://doi.org/10.4132/jptm.2019.09.16

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Abstract PDF Supplementary Material

Background
The worldwide incidence of squamous cell carcinoma of the tongue (SCCOT) in young patients has been increasing. We investigated clinicopathologic features of this unique population and compared them with those of SCCOT in the elderly to delineate its pathogenesis.
Methods
We compared clinicopathological parameters between patients under and over 45 years old. Immunohistochemical assays of estrogen receptor, progesterone receptor, androgen receptor, p53, p16, mdm2, cyclin D1, and glutathione S-transferase P1 were also compared between them.
Results
Among 189 cases, 51 patients (27.0%) were under 45 years of age. A higher proportion of women was seen in the young group, but was not statistically significant. Smoking and drinking behaviors between age groups were similar. Histopathological and immunohistochemical analysis showed no significant difference by age and sex other than higher histologic grades observed in young patients.
Conclusions
SCCOT in young adults has similar clinicopathological features to that in the elderly, suggesting that both progress via similar pathogenetic pathways.

Citations

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High Failure Rates in Young Nonsmoker Nondrinkers With Squamous Cell Carcinoma of the Oral Tongue
Brianna M. Jones, Dillan F. Villavisanis, Eric J. Lehrer, Daniel R. Dickstein, Kunal K. Sindhu, Krzysztof J. Misiukiewicz, Marshall Posner, Jerry T. Liu, Vishal Gupta, Sonam Sharma, Scott A. Roof, Marita Teng, Eric M. Genden, Richard L. Bakst
The Laryngoscope.2023; 133(5): 1110. CrossRef
Characteristics of oral squamous cell carcinoma focusing on cases unaffected by smoking and drinking: A multicenter retrospective study
Hiroyuki Harada, Masahiro Kikuchi, Ryo Asato, Kiyomi Hamaguchi, Hisanobu Tamaki, Masanobu Mizuta, Ryusuke Hori, Tsuyoshi Kojima, Keigo Honda, Takashi Tsujimura, Yohei Kumabe, Kazuyuki Ichimaru, Yoshiharu Kitani, Koji Ushiro, Morimasa Kitamura, Shogo Shino
Head & Neck.2023; 45(7): 1812. CrossRef
Genetic characteristics of advanced oral tongue squamous cell carcinoma in young patients
Sehui Kim, Chung Lee, Hyangmi Kim, Sun Och Yoon
Oral Oncology.2023; 144: 106466. CrossRef
Oral Squamous Cell Carcinoma Frequency in Young Patients from Referral Centers Around the World
Rafael Ferreira e Costa, Marina Luiza Baião Leão, Maria Sissa Pereira Sant’Ana, Ricardo Alves Mesquita, Ricardo Santiago Gomez, Alan Roger Santos-Silva, Syed Ali Khurram, Artysha Tailor, Ciska-Mari Schouwstra, Liam Robinson, Willie F. P. van Heerden, Rami
Head and Neck Pathology.2022; 16(3): 755. CrossRef
Early-onset oral cancer as a clinical entity: aetiology and pathogenesis
E.S. Kolegova, M.R. Patysheva, I.V. Larionova, I.K. Fedorova, D.E. Kulbakin, E.L. Choinzonov, E.V. Denisov
International Journal of Oral and Maxillofacial Surgery.2022; 51(12): 1497. CrossRef
The effect of age on the clinicopathological features of oral squamous cell carcinoma
Alaa S Saeed, Bashar H Abdullah
Journal of Baghdad College of Dentistry.2022; 34(1): 25. CrossRef
Survival Outcomes in Oral Tongue Cancer: A Mono-Institutional Experience Focusing on Age
Mohssen Ansarin, Rita De Berardinis, Federica Corso, Gioacchino Giugliano, Roberto Bruschini, Luigi De Benedetto, Stefano Zorzi, Fausto Maffini, Fabio Sovardi, Carolina Pigni, Donatella Scaglione, Daniela Alterio, Maria Cossu Rocca, Susanna Chiocca, Sara
Frontiers in Oncology.2021;[Epub] CrossRef
A meta-analysis of oral squamous cell carcinoma in young adults with a comparison to the older group patients (2014–2019)
Khadijah Mohideen, C Krithika, Nadeem Jeddy, Thayumanavan Balakrishnan, R Bharathi, SLeena Sankari
Contemporary Clinical Dentistry.2021; 12(3): 213. CrossRef
Modern perspectives of oral squamous cell carcinoma
A.A. Ivina
Arkhiv patologii.2020; 82(3): 55. CrossRef

Case Studies

WHO Grade IV Gliofibroma: A Grading Label Denoting Malignancy for an Otherwise Commonly Misinterpreted Neoplasm: Paola A. Escalante Abril, Miguel Fdo. Salazar, Nubia L. López García, Mónica N. Madrazo Moya, Yadir U. Zamora Guerra, Yadira Gandhi Mata Mendoza, Erick Gómez Apo, Laura G. Chávez Macías; J Pathol Transl Med. 2015;49(4):325-330. Published online June 17, 2015; DOI: https://doi.org/10.4132/jptm.2015.05.20; Correction in: J Pathol Transl Med 2015;49(6):538

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Abstract PDF

We report a 50-year-old woman with no relevant clinical history who presented with headache and loss of memory. Magnetic resonance imaging showed a left parieto-temporal mass with annular enhancement after contrast media administration, rendering a radiological diagnosis of high-grade astrocytic neoplasm. Tumour sampling was performed but the patient ultimately died as a result of disease. Microscopically, the lesion had areas of glioblastoma mixed with a benign mesenchymal constituent; the former showed hypercellularity, endothelial proliferation, high mitotic activity and necrosis, while the latter showed fascicles of long spindle cells surrounded by collagen and reticulin fibers. With approximately 40 previously reported cases, gliofibroma is a rare neoplasm defined as either glio-desmoplastic or glial/benign mesenchymal. As shown in our case, its prognosis is apparently determined by the degree of anaplasia of the glial component.

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Rare Pediatric Invasive Gliofibroma Has BRAFV600E Mutation and Transiently Responds to Targeted Therapy Before Progressive Clonal Evolution
Kristiyana Kaneva, Kee Kiat Yeo, Debra Hawes, Jianling Ji, Jaclyn A. Biegel, Marvin D. Nelson, Stefan Bluml, Mark D. Krieger, Anat Erdreich-Epstein
JCO Precision Oncology.2019; (3): 1. CrossRef

Anaplastic Transformation of Papillary Thyroid Carcinoma in a Young Man: A Case Study with Immunohistochemical and BRAF Analysis: Ji Hye Park, Hyeong Ju Kwon, Cheong Soo Park, SoonWon Hong; Korean J Pathol. 2014;48(3):234-240. Published online June 26, 2014; DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.3.234

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Abstract PDF

This study reports a case of anaplastic transformation from a well-differentiated thyroid carcinoma in a young patient. The first recurrent tissue contained poorly differentiated foci that revealed lower thyroglobulin, thyroid transcription factor 1 (TTF-1), and galectin-3 expression than the well-differentiated area. However there was no increased p53 or Ki-67 expression in the poorly differentiated foci, nor in the well-differentiated area. The tissue subsequently relapsed and revealed only anaplastic features, complete loss of thyroglobulin, TTF-1, and galectin-3 expression and revealed an increase in p53 and Ki-67 expression. The BRAF V600E and BRAF V600V mutation were found in the initially diagnosed papillary thyroid carcinoma and the poorly differentiated foci of the recurring papillary thyroid carcinoma; however, only the BRAF V600V mutation was found in the anaplastic carcinoma. These results suggest that overexpression of p53 and Ki-67 contributed to the anaplastic transformation. We also found that the BRAF type changed during the tumor relapse.

Citations

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Immunomodulation exerted by galectins: a land of opportunity in rare cancers
Laura Díaz-Alvarez, Georgina I. López-Cortés, Erandi Pérez-Figueroa
Frontiers in Immunology.2023;[Epub] CrossRef
Coexisting well-differentiated and anaplastic thyroid carcinoma in the same primary resection specimen: immunophenotypic and genetic comparison of the two components in a consecutive series of 13 cases and a review of the literature
Moira Ragazzi, Federica Torricelli, Benedetta Donati, Alessia Ciarrocchi, Dario de Biase, Giovanni Tallini, Eleonora Zanetti, Alessandra Bisagni, Elisabetta Kuhn, Davide Giordano, Andrea Frasoldati, Simonetta Piana
Virchows Archiv.2021; 478(2): 265. CrossRef
HDAC Inhibition Induces PD-L1 Expression in a Novel Anaplastic Thyroid Cancer Cell Line
Luca Hegedűs, Dominika Rittler, Tamás Garay, Paul Stockhammer, Ildikó Kovács, Balázs Döme, Sarah Theurer, Thomas Hager, Thomas Herold, Stavros Kalbourtzis, Agnes Bankfalvi, Kurt W. Schmid, Dagmar Führer, Clemens Aigner, Balázs Hegedűs
Pathology & Oncology Research.2020; 26(4): 2523. CrossRef
EWSR1 rearrangement is a frequent event in papillary thyroid carcinoma and in carcinoma of the thyroid with Ewing family tumor elements (CEFTE)
G. Oliveira, A. Polónia, J. M. Cameselle-Teijeiro, D. Leitão, S. Sapia, M. Sobrinho-Simões, C. Eloy
Virchows Archiv.2017; 470(5): 517. CrossRef
Anaplastic carcinoma of the thyroid in a 12-year old girl
Rodrigo Mon, James Newlon
Journal of Pediatric Surgery Case Reports.2015; 3(9): 404. CrossRef

Case Report

Primary Hepatic Neuroblastoma: A Case Report.: Geunyoung Jung, Jihun Kim; Korean J Pathol. 2011;45(4):423-427.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.4.423

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Abstract PDF

Neuroblastoma is a malignant tumor of primordial neural crest origin. It usually develops along the sympathetic nervous system, such as the adrenal glands or paramedian sympathetic chain and metastasizes to the liver most frequently. However, a primary hepatic neuroblastoma has not been reported yet. Here, we report a case of 29-year-old woman who presented with a solitary hepatic mass. Grossly, the mass was large, creamy, rubbery firm, and showed focal hemorrhage and central cavitation. Microscopically, the tumor cells were arranged in small nests of spindle to ovoid cells with abundant neuropil. The neuroblastic nature of the tumor was confirmed by immunohistochemistry and electron microscopy. No extrahepatic mass was found, despite a thorough systemic survey such as chest and abdominopelvic computed tomography (CT) scans and a whole body positron emission tomography-CT study. To the best of our knowledge, this is the first report of a bona fide primary hepatic neuroblastoma.

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Primary hepatic neuroblastoma in a 19-month-old child: A case report
Jovana Dimić, Dejan Skorić, Aleksandar Sretenović, Slaviša Đuričić
Medicinska istrazivanja.2022; 55(2): 41. CrossRef
Morphologic Alteration of Metastatic Neuroblastic Tumor in Bone Marrow after Chemotherapy
Go Eun Bae, Yeon-Lim Suh, Ki Woong Sung, Jung-Sun Kim
Korean Journal of Pathology.2013; 47(5): 433. CrossRef

Original Article

Vascular Endothelial Growth Factor Bioactivity and Its Receptors in Patients with Acute Respiratory Distress Syndrome.: Simona Gurzu, Ioan Jung, Leonard Azamfirei, Bong Young Shin, Raluca Solomon, Daria Demian, Judith Kovacs, Han Kyeom Kim; Korean J Pathol. 2011;45(2):139-145.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.2.139

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Abstract PDF

BACKGROUND
Pathogenesis of acute respiratory distress syndrome (ARDS) is a controversial issue. Few studies have analyzed the possible role of vascular endothelial growth factor (VEGF) and its receptors in this lesion.
METHODS
We compared the immunohistochemical expression of VEGF, its receptors (VEGFR1, VEGFR2) and CD68, in normal lungs and lungs with ARDS. Fifty necropsy cases and 12 lung biopsies with ARDS were analyzed. In total, eight cases were in the early stage and 54 cases were in late stage of ARDS. In addition, the serum level of VEGF165 was also determined.
RESULTS
In normal lungs, all antibodies marked the endothelial cells (EC) and pneumocytes (PC), except for CD68, which was expressed in the alveolar macrophages. In early ARDS, the intensity of VEGF165 and VEGFR2 decreased in both EC and PC. VEGF121 was absent in PC but its expression increased in bronchial epithelium. VEGFR1 was expressed in the integral PC. In late ARDS, VEGF165 down-regulation was more significant in PC and EC but its intensity increased in hyaline membranes (HM). In some cases, HM were CD68 positive. The serum level of VEGF165 was up-regulated, while VEGF165 intensity in PC decreased and the HM appeared in alveolar spaces.
CONCLUSIONS
Sporadic positivity of HM for CD68 and decreasing of VEGF165 expression in EC proved that VEGF165 is produced by PC, destroyed macrophages, and extravasated serum.

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Hallazgos similares al COVID-19 en un caso fatal de neumonía intersticial descamativa asociada con glomerulonefritis por IgA en una niña de 13 meses de edad
Simona Gurzu, Catalin-Bogdan Satala, Lorena Elena Melit, Adrian Streinu-Cercel, Dan Otelea, Brandusa Capalna, Claudiu Ioan Puiac, Janos Szederjesi, Ioan Jung
Kompass Neumología.2021; 3(2): 69. CrossRef
COVID-19 Like Findings in a Fatal Case of Idiopathic Desquamative Interstitial Pneumonia Associated With IgA Glomerulonephritis in a 13-Month-Old Child
Simona Gurzu, Catalin Bogdan Satala, Lorena Elena Melit, Adrian Streinu-Cercel, Dan Otelea, Brandusa Capalna, Claudiu Ioan Puiac, Janos Szederjesi, Ioan Jung
Frontiers in Pediatrics.2020;[Epub] CrossRef

Case Reports

Clear Cell Sarcoma of the Kidney: A case in 39 year old man.: Hyun Ju Yoo, Yun Kyung Kang, Mee Joo, Hye Kyung Lee, Dae Woo Kim, Suk San Park; Korean J Pathol. 1996;30(12):1138-1143.

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Abstract PDF: Clear cell sarcoma of kidney(CCSK) is a rare pediatric neoplasm characterized by a predominating component of clear cells, a predilection for metastases to bone, and a poor prognosis. The incidence of CCSK peaks during the 2nd year of life and adult cases are very rare. We report a case of CCSK encountered in the right kidney of a 39-year-old man. Grossly, it was a lobulated mass showing infiltrative margin, measured 7x5.5x5cm and had a homogeneous gray-tan color with a soft, fish-flesh consistency. Microscopically, about half of the tumor revealed the classic pattern of CCSK, having tumor cell cords or nests separated by the characteristic alveolar capillary networks. The tumor cells had clear pale cytoplasm, bland looking round nuclei and inconspicuous nucleoli. The other half showed the epithelioid-trabecular pattern forming pseudorosette or cord-like structures. Immunohistochemically, there was only a focal positive reaction to vimentin. Ultrastructurally, the tumor cells showed the primitive nephrogenic mesenchymal differentiation such as electron lucent cytoplasm, a small amount of organelles, scanty heterochromatin, inconspicuous nucleoli, and a lack of flocculant basal lamina material around the cytoplasmic membrane. We consider that this is a case of CCSK occuring in the oldest patient ever reported, confirmed by both immunohistochemistry and electron microscopy.

Potter's Syndrome with Adult Polycystic Renal Disease: An autopsy case report.: Hwa Sook Jeong, Beom Soo Park, Geon Kook Lee; Korean J Pathol. 1997;31(4):361-365.

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Abstract PDF: Potter's syndrome including bilateral renal agenesis or polycystic renal disease, bilateral pulmonary hypoplasia and characteristic face was first described in 1946. Although a great number of cases of Potter's syndrome was reported, Potter's syndrome with adult polycystic kidney disease(Potter type III) was very rarely found. In this report, we described an autopsy case of Potter's syndrome having adult polycystic kidneys disease, bilateral pulmonary hypoplasia and characteristic face in conjunction with multiple hepatic cysts, features of congenital hepatic fibrosis and a pancreatic cyst. Microscopically, all cysts were lined by cuboidal epithelial cells, showing positive for epithelial membrane antigen and cytokeratins.

Adenocarcinoma Arising in Sacrococcygeal Teratoma: A case report.: Hae Jeong Choi, Mi Jin Gu, Yeong Kyung Bae, Joon Hyuk Choi, Jae Hwan Kim; Korean J Pathol. 1998;32(4):315-317.

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Abstract PDF: We experienced a case of adenocarcinoma arising in sacrococcygeal teratoma. The patient was a 52-year-old woman. She was admitted due to one month of sacral pain. She had a sacral mass since birth. On physical examination, anal fistula was present at the perianal area and pus drainage was noted. MR image showed multiple variable-sized cysts with inhomogeneous density. Resected specimen, mesuring 12.5 7.0 cm in diameter, showed multiple variable-sized cystic lesions admixed with grayish solid portion. The cysts contained mucoid material. The microscopic examination showed mature teratoma composed of cysts lined by pseudostratified ciliated columnar epithelium, intestinal mucosa, mature cartilage, bone, and fat tissue. A moderately differentiated adenocarcinoma developed from the cystic area in the mass.

Malignant Rhabdoid Tumor of the Cerebellum in an Adult: A case report.: Young Min Kim, Jae Hee Suh, Tae Sook Kim, Shin Kwang Khang; Korean J Pathol. 1998;32(6):460-465.

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Abstract: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive neoplasm of infancy and childhood. Although it was originally described and most frequently reported in the kidney, it may occur in various extra-renal sites such as the liver, thymus, and soft tissue. In the last decade primary central nervous system (CNS) MRTs have been reported in both the supra- and infratentorial compartments. Patients with CNS MRT were generally below the age of two and reports in adults are extremely rare. This is a case of primary cerebellar MRT in a 24-year-old woman, who had presented with intermittent headache, vocal cord palsy, and cerebellar dysfunctions such as abnormal finger to nose test and tandem gait. By magnetic resonance imaging scan, a well-enhancing solid mass was demonstrated at the posterior fossa filling the 4th ventricle, which extended into the medulla and cervical cord via the foramen of Magendie. Histologically, the monotonous polygonal tumor cells were arranged in diffuse sheet with occasional hemorrhagic necrosis. The nuclei were vesicular and eccentrically located due to eosinophilic, PAS-positive, intracytoplasmic inclusions with prominent nucleoli. They were diffusely or focally immunoreactive for vimentin, neurofilament, cytokeratin, GFAP, synaptophysin, and smooth muscle actin, while epithelial membrane antigen and desmin were negative. Ultrastructurally, the polyhedral tumor cells were densely packed with primitive intercellular junctions. Scanty fibrillar intermediate filaments were intermingled with cellular organelles. Postoperatively, craniospinal irradiation and systemic chemotherapy have been done and she has been free of tumor recurrence during the 13 months' follow-up periods.

Gastric Duplication Associated with Marked Atypism: A case report.: Hae Joo Nam; Korean J Pathol. 1999;33(1):52-54.

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Abstract PDF: A case of gastric duplication associated with marked atypism in an adult is reported. The patient was a 62-year-old woman presenting a mass in left upper quadrant of abdomen. The mass was a closed gastric cyst located in the greater curvature, measuring 11 9 cm in diameter. The cyst had common muscular layer with stomach. It showed gray-white firm cystic wall with yellowish brown soft necrotic tissue. Microscopically, the cyst was lined by columnar mucin-secreting epithelium having marked cellular atypism. The cyst wall was supported by layers of smooth muscle, accompanied with severe fibrosis and chronic inflammation.

Original Articles

Comparison of Pathologic Findings of Cortical Lobectomy for Intractable Seizures between Children and Adults: An Analysis of 164 Cases.: Na Rae Kim, Yeon Lim Suh; Korean J Pathol. 1999;33(12):1175-1181.

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Abstract PDF: Many pathological surveys of brain tissue in patients with intractable epilepsy have been reported. There have been, however, few studies focused on the differences between childhood and adults in pathological alterations of brain. We retrospectively analyzed histopathology of 164 lobectomy specimens for intractable epilepsy in view of the differences between children and adults. Among 164 cases, 28 cases were children (less than 15 years) and 136 cases adults. We compared frequency of histopathologic features, distribution of involved cortex (temporal or extratemporal lobe), previous injury histories, such as brain trauma, encephalitis or febrile seizure, and coexistence of other lesions (dual pathology) between two groups. Pathologic alterations were encountered in 92% of 164 patients. In children focal cortical dysplasia (n=16, 57.1%), neoplasm (n=8, 28.6%), hippocampal sclerosis (n=6, 21.4%), cortical tuber (n=1, 3.6%), leukomalacia (n=1, 3.6%), and Rasmussen's encephalitis (n=1, 3.6%) were observed, whereas focal cortical dysplasia (n=81, 59.6%), hippocampal sclerosis (n=80, 58.8%), neoplasm (n=19, 14%), and cerebral cysticercosis (n=3, 2.2%) were found in adults. Pediatric patients had a higher proportion of severe focal cortical dysplasia (17.9% in children, 0.7% in adults). Neoplasia and extratemporal lobe involvement were more commonly found in children (28.6%, 50%) than in adults (14.0%, 24.3%), whereas hippocampal sclerosis and dual pathology were more common in adults (58.8%, 44.9%) than in children (21.4%, 17.9%). Previous injury history was statistically significant in patients with hippocampal sclerosis, and lent support to the hypothesis that hippocampal sclerosis is related with acquired lesions. Incidence of focal cortical dysplasia was nearly similar in both adult (59.6%) and pediatric groups (57.1%), and supported the hypothesis that focal cortical dysplasia is developmental abnormality occurring during a prenatal period.

Juvenile Hyaline Fibromatosis in an Adult.: Young A Kim, Seoung Wan Chae, Chong Jai Kim, Je G Chi; Korean J Pathol. 2000;34(3):239-242.

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Abstract PDF: Juvenile hyaline fibromatosis is a rare disorder probably inherited as an autosomal recessive trait. It is characterized by multiple slowly growing subcutaneous nodules, hypertrophy of gingiva, flexion contracture, and radiolucent bone destruction. The histological features of the tumor-like lesions are characterized by the deposition of amorphous hyaline material in which spindle shaped cells are embedded. We report a case of juvenile hyaline fibromatosis in a 26 year-old-woman. She had multiple subcutaneous nodules in scalp, ear, forearms, right knee, and back. Surgical excision of the tumors in the scalp and ear was done. The largest one measured 13 9 6 cm, and had homogeneous, grayish yellow cut surface with calcification. Light microscopic examination showed abundant eosinophilic hyaline material with extensive calcification and metaplastic bone formation. Spindle cells were rarely observed at the periphery of the tumor. Hyaline matrix was PAS positive, diastase resistant, and alcian blue negative. Scattered spindle cells were positive for vimentin but negative for S-100 protein and smooth muscle actin. There were many reports regarding early lesions of juvenile hyaline fibromatosis; however in this patient, tumor existed for more than 20 years and the histology was somewhat different from the early lesions reported in the literature.

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