Journal of Pathology and Translational Medicine

Original Articles

A Proposal for Creating a Guideline for Cancer Registration of the Fibromatosis, PEComa Group, Malignant Lymphoma In Situ and Dendritic Cell Tumors (III): Changyoung Yoo, Chang Suk Kang, Yoon La Choi, Hye Yoon Kang, Jin Man Kim, Young Hye Koh, Joo Hee Lee, Seung Sook Lee, In Sun Kim, Dong Hoon Kim, Yong Ku Park, Jin Hee Sohn; Korean J Pathol. 2012;46(5):436-442. Published online October 25, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.5.436

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Abstract PDF: Background
Understanding the biologic behavior of a tumor is a prerequisite for tumor registration code assignment. The aim of this report was to propose appropriate behavior codes of the International Classification of Disease Oncology 3 (ICD-O3) to rare, yet pathologically interesting hematopoietic and soft tissue tumors.
Methods
The Study Group for Hematopathology, the Bone and Soft Tissue Pathology Study Group, and the Cancer Registration Committee prepared the questionnaire containing provisional behavior codes of selected diseases.
Results
In situ lesions of mantle cell and follicular lymphomas, dendritic cell tumors, and neoplasms with perivascular epithelioid cell differentiation (PEComa), not otherwise specified were classified as malignant (-/3). The fibromatosis group, with the exception of lipofibromatosis, was proposed as benign (-/0). Lipofibromatosis and several diseases that belong to the PEComa group were proposed as uncertain malignant potential (-/1). For the hematologic and soft tissue tumors, 274 and 288 members of the Korean Society of Pathologists, respectively, provided opinions through questionnaire, and most responders showed agreement with the provisional behavior code proposed.
Conclusions
The determination of behavior codes for the rare diseases described in this study, especially those of the PEComa group or malignant lymphoma, could be viewed as impractical and premature, but this study provides the basis for future research on this topic.

Proposal for Creating a Guideline for Cancer Registration of Microinvasive Tumors of the Breast and Ovary (II): Jin Hee Sohn, Gyungyub Gong, Kyu Rae Kim, Chang Suk Kang, Youn Soo Lee, Jin Man Kim, Woo Hee Jung, Kwang Sun Suh; Korean J Pathol. 2012;46(3):226-232. Published online June 22, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.3.226

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Background

Cancer registration in Korea has a longer than 30-years of history, during which time cancer registration has improved and become well-organized. Cancer registries are fundamental for cancer control and multi-center collaborative research. However, there have been discrepancies in assigning behavior codes. Thus, we intend to propose appropriate behavior codes for the International Classification of Disease Oncology, 3rd edition (ICD-O-3) for microinvasive tumors of the ovary and breast not only to improve the quality of the cancer registry but also to prevent conflicts.

Methods

As in series I, two pathology study groups and the Cancer Registration Committee of the Korean Society of Pathologists (KSP) participated. To prepare a questionnaire on provisional behavior code, the relevant subjects were discussed in the workshop, and consensus was obtained by convergence of opinion from members of KSP.

Results

Microinvasive tumor of the breast should be designated as a microinvasive carcinoma which was proposed as malignant tumor (/3). Serous borderline tumor with microinvasion of the ovary was proposed as borderline tumor (/1), and mucinous borderline tumor with microinvasion of the ovary as either borderline (/1) or carcinoma (/3) according to the tumor cell nature.

Conclusions

Some issues should be elucidated with the accumulation of more experience and knowledge. Here, however, we present our second proposal.

Citations

Citations to this article as recorded by

Update on the Proposal for Creating a Guideline for Cancer Registration of the Gastrointestinal Tumors (I-2)
Eun Sun Jung, Yun Kyung Kang, Mee-Yon Cho, Joon Mee Kim, Won Ae Lee, Hee Eun Lee, Sunhoo Park, Jin Hee Sohn, So-Young Jin
Korean Journal of Pathology.2012; 46(5): 443. CrossRef
A Proposal for Creating a Guideline for Cancer Registration of the Fibromatosis, PEComa Group, Malignant LymphomaIn Situand Dendritic Cell Tumors (III)
Changyoung Yoo, Chang Suk Kang, Yoon La Choi, Hye Yoon Kang, Jin Man Kim, Young Hye Koh, Joo Hee Lee, Seung Sook Lee, In Sun Kim, Dong Hoon Kim, Yong Ku Park, Jin Hee Sohn
Korean Journal of Pathology.2012; 46(5): 436. CrossRef

Case Report

Sclerosing Polycystic Adenosis of the Parotid Gland: A Case Report.: Byung Joo Jeong, Mi Ran Kim, Zhe Long Liang, Bon Seok Koo, Jin Man Kim; Korean J Pathol. 2011;45:S79-S83.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S79

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Abstract PDF

Sclerosing polycystic adenosis (SPA) of the salivary glands is a rare entity analogous to fibrocystic disease of the breast. Less than 50 cases of SPA have been published in the literature. We report the first Korean case of SPA of the right parotid gland. A 34-year-old man presented with a slowly growing right parotid mass. Computed tomography showed a relatively well-demarcated, heterogeneously enhancing mass with multiple small calcifications. Fine needle aspiration showed cohesive sheets of epithelial cells with granular oncocytic cytoplasm and scattered lymphocytes. The parotidectomy specimen showed a 3 cm-sized solid nodular lesion with small cysts. Microscopically, the lesion was an unencapsulated mass of sclerotic fibrous tissue with cystic ducts, multiple calcifications, and lymphoplasma cell infiltration. Sclerosing adenosis and cystic ducts with frequent apocrine-like cells were noted. Familiarity with the cytologic and histological features of SPA is very important making the correct diagnosis.

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A Case of Sclerosing Polycystic Adenosis of Parotid Gland
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Original Article

WHO Classification of Malignant Lymphomas in Korea: Report of the Third Nationwide Study.: Jin Man Kim, Young Hyeh Ko, Seung Sook Lee, Jooryung Huh, Chang Suk Kang, Chul Woo Kim, Yun Kyung Kang, Jai Hyang Go, Min Kyung Kim, Wan Seop Kim, Yoon Jung Kim, Hyun Jung Kim, Hee Kyung Kim, Jong Hee Nam, Hyung Bae Moon, Chan Kum Park, Tae In Park, Young Ha Oh, Dong Wha Lee, Jong Sil Lee, Juhie Lee, Hyekyung Lee, Sung Chul Lim, Kyu Yun Jang, Hee Kyung Chang, Yoon Kyung Jeon, Hye Ra Jung, Min Sun Cho, Hee Jeong Cha, Suk Jin Choi, Jae Ho Han, Sook Hee Hong, Insun Kim; Korean J Pathol. 2011;45(3):254-260.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.3.254

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Abstract PDF

BACKGROUND
The aim of study was to determine the relative frequency of malignant lymphoma according to World Health Organization (WHO) classification in Korea.
METHODS
A total of 3,998 cases diagnosed at 31 institutes between 2005 and 2006 were enrolled. Information including age, gender, pathologic diagnosis, site of involvement and immunophenotypes were obtained.
RESULTS
The relative frequency of non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) was 95.4% and 4.6%, respectively. B-cell lymphomas accounted for 77.6% of all NHL, while T/natural killer (T/NK)-cell lymphomas accounted for 22.4%. The most frequent subtypes of NHL were diffuse large B-cell lymphoma (42.7%), extranodal marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue (19.0%), NK/T-cell lymphoma (6.3%) and peripheral T-cell lymphoma (PTCL), unspecified (6.3%), in decreasing order. The relative frequency of HL was nodular sclerosis (47.4%), mixed cellularity (30.6%), and nodular lymphocyte predominant (12.1%) subtypes. Compared with a previous study in 1998, increase in gastric MZBCL and nodular sclerosis HL, and slight decrease of follicular lymphoma, PTCL, and NK/T-cell lymphoma were observed.
CONCLUSIONS
Korea had lower rates of HL and follicular lymphoma, and higher rates of extranodal NHL, extranodal MZBCL, and NK/T-cell lymphoma of nasal type compared with Western countries. Changes in the relative frequency of lymphoma subtypes are likely ascribed to refined diagnostic criteria and a change in national health care policy.

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CD44s and CD44v6 Are Predominantly Expressed in the Non-germinal Center B-Cell-like Type of Diffuse Large B-Cell Lymphomas
Kyueng-Whan Min, Young-Ha Oh, Chan-Kum Park, So-Dug Lim, Wan-Seop Kim
The Korean Journal of Pathology.2011; 45(6): 589. CrossRef

Case Report

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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Sclerosing angiomatoid nodular transformation of the spleen presenting rapid growth after adrenalectomy: Report of a case
Yasuo Nagai, Daisuke Satoh, Hiroyoshi Matsukawa, Shigehiro Shiozaki
International Journal of Surgery Case Reports.2017; 30: 108. CrossRef
A Case of Sclerosing Angiomatoid Nodular Transformation of the Spleen: Spoke Wheel Pattern on Computed Tomography
Joo Han Park, Sun Hyuk Hwang, Ki Chan Kim, Si Yeon Kim, Ga Won Song, Jae Ho Han, Joon Seong Park
Korean Journal of Medicine.2014; 86(3): 357. CrossRef
Differentiation of an Unclear Splenic Lesion in a Patient With Cholangiocarcinoma
Constantin Lapa, Ulrich Steger, Christian O. Ritter, Vanessa Wild, Ken Herrmann
Clinical Nuclear Medicine.2014; 39(5): 470. CrossRef
Sclerosing Angiomatoid Nodular Transformation of the Spleen: CT and MRI Features With Pathologic Correlation
Rachel B. Lewis, Grant E. Lattin, Meenakshi Nandedkar, Nadine S. Aguilera
American Journal of Roentgenology.2013; 200(4): W353. CrossRef

Original Articles

Korean Pediatric/Adolescent Lymphoma: Incidence and Pathologic Characteristics.: Seung Sook Lee, Jin Man Kim, Young Hyeh Ko, Jooryung Huh, Chang Suk Kang, Chul Woo Kim, Yun Kyung Kang, Jai Hyang Go, Min Kyung Kim, Wan Seop Kim, Yoon Jung Kim, Hyun Jung Kim, Hee Kyung Kim, Jong Hee Nam, Hyung Bae Moon, Chan Kum Park, Tae In Park, Young Ha Oh, Dong Wha Lee, Jong Sil Lee, Juhie Lee, Hyekyung Lee, Sung Chul Lim, Kyu Yun Jang, Hee Kyung Chang, Yoon Kyung Jeon, Hye Ra Jung, Min Sun Cho, Hee Jeong Cha, Suk Jin Choi, Jae Ho Han, Sook Hee Hong, Insun Kim; Korean J Pathol. 2010;44(2):117-124.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.2.117

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Abstract PDF

BACKGROUND
The Hematopathology Study Group of the Korean Society of Pathologists conducted a nation-wide retrospective analysis of Korean pediatric lymphoma, to provide pathologic data on pediatric/adolescent lymphoma subtypes and features.
METHODS
All lymphoma cases of all age groups were collected during a recent 2 year-period (2005-2006) from 32 institutes in Korea. Among 3,686 lymphoma patients, 142 who were age 18 or less were classified according to the World Health Organization (WHO) classification.
RESULTS
Among 142 pediatric/adolescent lymphoma patients, Hodgkin lymphoma accounted for 21 (14.8%) and non-Hodgkin lymphoma (NHL) for 121 (85.2%). Hodgkin lymphoma appears to be more common in the pediatric/adolescent age group than in the all-ages group (14.8% vs 4.4%). T- and natural killer cell-NHL was more common in the pediatric/adolescent age group than in the all ages group (46.3% vs 22%). The majority of Korean pediatric/adolescent NHL cases was composed of Burkitt lymphoma, T- or B-lymphoblastic lymphoma, anaplastic large-cell lymphoma, and diffuse large B-cell lymphoma. For lymphoma patients under the age of 6 years, most had B-lymphoblastic or Burkitt lymphoma, which commonly presented at extranodal sites.
CONCLUSIONS
The distribution of lymphoma subtypes in the pediatric/adolescent age group is quite different from the distribution of adults, but it was quite similar to distribution in Western countries.

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A Case of Nasal Type Extranodal NK/T Cell Lymphoma Incidentally Detected in a Child
Ha Jin Oh, Sang Hyun Park, Hae In Jang, Dong Hoon Lee, Yoo Duk Choi, Hee Jo Baek, Hoon Kook
Clinical Pediatric Hematology-Oncology.2015; 22(1): 76. CrossRef

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.

Analyses of Genetic Alterations in Breast Cancers by Comparative Genomic Hybridization.: Jin Man Kim, Young Mi Jeon, Young Hyeh Ko, Kyu Sang Song, Howe J Ree, Joo Seob Keum, Jae Hyuk Lee, Sun Hoe Koo; Korean J Pathol. 1999;33(8):603-613.

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Abstract PDF: Transformation and progression of breast cancer are thought to be caused by an accumulation of complex genetic alterations, but little is known about specific changes. In this study, the author has undertaken a genome-wide screening to detect genetic changes in 20 cases of breast cancer among Koreans, including 16 infiltrating ductal carcinomas, 2 medullary carcinomas, 1 invasive lobular carcinoma, and 1 borderline phyllodes tumor. Comparative genomic hybridization (CGH) was used to screen for DNA sequence gains and losses across all human chromosomes. Simultaneous immunohistochemical staining for c-erbB-2 (Her-2/neu), c-myc, cyclin D1, and p53 protein was done to make comparisons with nuclear grade and that with CGH results. Biotin-labeled tumor DNA and digoxigenin-labeled normal DNA were hybridized to normal metaphase cells. The fluorescence signals were captured by fluorescence microscope after detection by avidin-FITC and anti-digoxigenin rhodamine. Then, the ratio of fluorescence was calculated by an image analyzer. The immunohistochemical staining was done in paraffin-embedded tissue with an LSAB kit and avidin-biotin complex (ABC) method. The CGH results showed gains on chromosomes 8q (40%), 1q (30%), 17q (15%), 20q (15%), 18q (15%), 5p (15%), and 13q (15%). Deletions were on chromosomes 17p (45%) and 22q (20%). High-level amplifications (green/red ratio >1.5) were noted on chromosomes 1p31, 1q, 3q25-qter, 5p, 7q31-qter, 8q, 9p22-qter, 10p, 11p, 11q22-qter, 12p, 12q24, 14q21-qter, 15q23-qter, 17q, 18p, 18q12-qter, 20p, and 20q. By comparison with infiltrating ductal carcinoma, the two medullary carcinomas showed high-level amplification on chromosomes 1p31, 1q, 8q, 10p, 11p and 12p. c-erbB-2, c-myc, cyclin D1, and p53 protein expression was immunohistochemically detected in 9 of 20 (45%), 8 of 20 (40%), 10 of 20 (50%), and 13 of 20 (65%), respectively. The results indicate that the amplification on chromosome 8q, 1q and the deletions on chromosomes 17p and 22q are the most frequent genetic alterations in breast cancers among Koreans. The results reveal a different pattern of genetic alteration from previous studies. The CGH results were not correlated with the immunohistochemical profiles. The amplification pattern of medullary carcinomas was quite different from the pattern of infiltrating ductal carcinomas. The CGH was thought to be very useful in the screening of genetic alterations of solid tumors.

Case Reports

Fine Needle Aspiration Cytology of Endodermal Sinus Tumor of the Ovary: A Case Report.: Jin Man Kim, Dae Young Kang; Korean J Cytopathol. 1995;6(1):54-57.

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Abstract PDF: We report a case of fine needle aspiration cytology of endodermal sinus tumor of the ovary. A 13-year-old girl complained of abdominal mass and pain. The abdominal sonography revealed a well-demarcated huge mass, which was solid and multiseptated. Percutaneous fine needle aspiration was performed from the mass. The smears revealed moderate cellularity, which was arranged in sheets or clumps of pleomorphic malignant cells on mucoid background. The valuable characteristic features of tumor cells were papillary configuration, vacuolated cytoplasm and intracellular and extracellular hyaline globules. The diagnosis was confirmed later by histologic study of surgical resection of the specimen.

Carcinosarcoma (True Malignant Mixed Tumor) of the Parotid Gland: A Report of a Case with Small Cell Carcinoma as the Carcinoma Component.: Hun Soo Kim, Hyang Jung Cho, Yeun Tai Chung, Soon Ah Park, Hae Joong Cho, Jin Man Kim; Korean J Pathol. 2008;42(3):175-180.

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Abstract PDF: True malignant mixed tumor (carcinosarcoma) of the salivary gland is an extremely rare tumor. By definition, it is a heterologous neoplasm that's composed of both malignant epithelial and mesenchymal components. We report here on a case of an 83 year old female patient with a parotid gland mass. Histologic examination showed a biphasic pattern of growth with both a poorly differentiated epithelial component and a sarcomatous spindle cell background. The immunohistochemical and ultrastructural findings support the diagnosis of a small cell neuroendocrine epithelial tumor and an undifferentiated malignant mesenchymal features. The histologic and ultrastructural features along with the immunohistochemical findings are presented. We also review the literature and we discuss the different opinions on the exact histogenesis of the true malignant mixed tumor of the salivary gland. To the best of our knowledge, this histological pattern has not been previously reported in the English medical literature.

Scleroma of the Larynx: A Case Report.: Hun Soo Kim, Seok Won Park, Jin Man Kim; Korean J Pathol. 2003;37(4):287-290.

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Abstract PDF: Scleroma of the larynx is a rare specific granulomatous disease and is usually presented as alaryngeal extension of a primary rhinoscleroma. We report an unusual case of laryngeal scleromain a 64-year-old female patient whose presented with progressive upper respiratory obstructivesymptoms lasted for 2 years. Symptoms were acutely exaggerated during the course of one month.On physical examination, the nasal mucosa was atrophic and crusted, and septal perforationwas noted. The fiberoptic laryngoscopy revealed a yellowish granulated epiglottic mass with adirty surface mimicking laryngeal cancer. Histological sectioning showed the characteristic featuresof scleroma including Mikulicz cells with scattered plasma cells and some neutrophils.Warthin-starry stains and electron microscopy examination showed numerous short rods insidethe cytoplasm of the Mikulicz cells. After the diagnosis, the patient underwent an emergency tracheostomyfollowed by long-term antibiotic treatment, and her symptoms have slowly improved.

High-grade Transformation of Primary Nodal Marginal Zone B-Cell Lymphoma: A Case Report.: Joo Heon Kim, Dong Wook Kang, Mee Ja Park, Jin Man Kim; Korean J Pathol. 2003;37(4):282-286.

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Abstract PDF: Primary nodal marginal zone B-cell lymphoma (MZBCL) is recognized as a rare and distinct entity. The rate of histologic transformation into diffuse large B-cell lymphoma (DLBCL) seems lower than the rate of transformation in follicular lymphoma. We herein report a rare case ofnodal MZBCL showing transformation into DLBCL. The patient was a 73-year-old female withcervical lymphadenopathy. On the initial biopsy, the lymph node architecture was diffuselyeffaced with an extensive interfollicular and parafollicular infiltrate of monocytoid B-cells. Therewere scattered large blastic B-cells without formations of compact sheets. The diagnosis ofnodal MZBCL was made. The patient did not receive chemotherapy and was treated with aconservatively supportive regimen. Forty two months later, the patient developed a new cervicallymphadenopathy and a biopsy was performed. Histologically, the lymph node revealeddiffuse sheets of transformed large B-cells showing prominent nucleoli. The diagnosis ofDLBCL transformed from nodal MZBCL was made. The patient was treated with 3 cycles ofcombined CHOP chemotherapy and she showed clinical improvement. These observationssuggest that an untreated primary nodal MZBCL may undergo high-grade transformation.

Original Article

Flow Cytometric DNA Analysis in Thyroid Neoplasms: With Emphasis on the Correlation between Ploidy Level and Pathologic Features.: Young Tae Kim, Jin Man Kim, Kwang Sun Suh, Jin Sun Bae; Korean J Pathol. 1995;29(2):127-135.

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Abstract PDF: Nuclear DNA content in 31 cases of thyroid neoplasm was determined by flow cytometry with the use of paraffin-embedded archival tissue. DNA aneuploidy was found in 6 cases (19.4%) of the 31 thyroid neoplasms; such as 2 of 8 (DI=1.16, 1.56) follicular adenomas, 1 of 6 (DI=1.10) follicular carcinomas, 1 of 15 (DI=1.18) papillary carcinomas and 2 of 2 (DI=1.76, 2.07) medullary carcinomas. The remaining tumors were diploid. No significant difference between follicular adenomas and carcinomas was detected with respect to the S phase fraction(SPF). In the papillary carcinoma group the SPF was higher than in the follicular neoplasm group, but it was statistically insignificant. Regional lymph node metastasis was present in 8 of 15( 53.3%) papillary carcinomas but absent in all of the 14 follicular neoplasms. In the medullary carcinoma group one case showed regional node metastasis at the time of resection and the other developed metastasis 11 months after surgical removal of the primary lesion. In this study tumors predominantly composed of Hurthle cells were found to have a significantly higher D.I. than those with few or no Hurthle cells. No significant difference was found between tumors with metastasis and those without metastasis.

Case Reports

Malignant Mixed Germ Cell Tumor and Contralateral Gonadoblastoma in Turner's Syndrome, 45, X0/46, XY Karyotype: A case report.: Dong Wook Kang, Jin Man Kim, Kwang Sun Suh, Kyu Sang Song, Dae Yung Kang; Korean J Pathol. 1995;29(1):85-90.

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Abstract PDF: Turner's syndrome results from complete or partial monosomy of the X chromosome and is characterized by hypogonadism or related other congenital anomalies in phenotypic females. In these patients, there are failure to develop normal secondary sex characteristics, amenorrhea, or short stature at puberty and the ovaries are reduced to atrophic fibrous strands devoid of ova and follicles(streak gonads). Individuals with this condition are particularly prone to the development of gonadoblastoma. For this reason, the gonads should be early removed and supplemental estrogen therapy given. We experienced a case of Turner's syndrome, 45, XO/46, XY karyotype in a 20-year-old phenotypic female complained an amenorrhea. On the exploratory laparotomy, the right gonadal mass is sevearly adhered to the adjacent organs and measures 8 x 5 x 5 cm in dimension and 75gm in weight and shows multiple foci of hemorrhage with necrosis. The left streak gonad measures 3.5 x 2 x 1.5 cm in dimension and shows multiple foci of calcification. Microscopically, the right gonadal mass reveals malignant mixed germ cell tumor, composed of endodermal sinus tumor, composed of endodermal sinus tumor with dysgerminoma and gonadoblastoma. The left streak gonad consists of mainly dense fibrous connective tissue and shows some foci of calcification associated with gonadoblastoma. On immunohistochemical and special stainings, the cytoplasm and hyalin droplets of the endodermal sinus tumor component reveal strong positivity to the a-fetoprotein and PAS. After removal of both gonads, the serum level of the a-fetoprotein is markedly down from 1742ng/ml to 2.6 ng/ml.

Atypical Mesoblastic Nephroma: Report of a case.: Jin Man Kim, Dong Wook Kang, Seung Ki Min, Kwang Sun Suh, Dae Young Kang; Korean J Pathol. 1991;25(6):601-606.

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Abstract PDF: Congenital mesoblastic nephroma(CMN) is an important differential diagnosis of a renal mass occurring in the newborn or in early childhood. It was first described by Bolande as a separate disease entity distinct form Wilms' tumor. In 1974, Beckwith has predicted that this tumor has a pathologic spectrum with classic congenital mesoblastic nephroma at one extreme, unequivocally mallignant spindle cell sarcomas at the other, and intermediate "gray zone" lesions of indeterminate biologic significanse. In 1986, Joshi has described "atypical mesoblastic nephroma" as a potentially aggressive variant of CMN, which shows atypical gross and microscopic features such as hemorrhage, necrosis, high cellularity, and mitotic index. We report of a case of atypical mesoblastic nephroma presenting in a 38 days-old male infant. Grossly, the tumor involved the upper and midportion of the left kidney. On section, the cut surface was fleshy, grayish-white, and homogeneous. Microscopically, the tumorshowed high degree of cellularity and arrangement of fusiform cells in sheets and vague interlacing bundles. The individual tumor cells showed fusiform to oval nuclei, indistinct scanty pale-eosinophilic cytoplasm and many mitotic figures.

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