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Original Articles
Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases
Dae Hyun Song, In Ho Choi, Sang Yun Ha, Kang Min Han, Jae Jun Lee, Min Eui Hong, Yoon-La Choi, Kee-Taek Jang, Sang Yong Song, Chin A Yi, Joungho Han
Korean J Pathol. 2014;48(3):188-192.   Published online June 26, 2014
DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.3.188
  • 7,737 View
  • 58 Download
  • 9 Crossref
AbstractAbstract PDF
Background

Lymphangioleiomyomatosis (LAM) is a slowly progressive neoplastic disease that predominantly affects females. Usually, LAM affects the lung; it can also affect extrapulmonary sites, such as the mediastinum, the retroperitoneum, or the lymph nodes, although these locations are rare. A localized form of LAM can manifest as extrapulmonary lesions; this form is referred to as extrapulmonary lymphangioleiomyoma (E-LAM). Due to the rare occurrence of E-LAM and its variable, atypical location, E-LAM is often difficult to diagnose. Herein, we report the clinicopathological information from four E-LAM cases, and also review previous articles investigating this disease.

Methods

Four patients with E-LAM were identified at the Samsung Medical Center (Seoul, Korea) from 1995 to 2012. All E-LAM lesions underwent surgical excision.

Results

All patients were females within the age range of 43 to 47 years. Two patients had para-aortic retroperitoneal masses, while the other two patients had pelvic lesions; two out of the four patients also had accompanying pulmonary LAM. In addition, no patient displayed any evidence of tuberous sclerosis. Histologically, two patients exhibited nuclear atypism with cytologic degeneration.

Conclusions

E-LAM should be considered in the differential diagnosis of patients presenting with pelvic or para-aortic masses. We also conclude that further clinical and pathological evaluation is needed in patients with E-LAM and nuclear atypism.

Citations

Citations to this article as recorded by  
  • Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
    Aleksandra Marciniak, Jolanta Nawrocka-Rutkowska, Agnieszka Brodowska, Andrzej Starczewski, Iwona Szydłowska
    Journal of Personalized Medicine.2023; 13(11): 1598.     CrossRef
  • A case of lymphangioleiomyomatosis with endometrial cancer diagnosed by retroperitoneoscopic para-aortic lymph node dissection
    Aiko Ogasawara, Shogo Yamaguchi, Hiroaki Inui, Mieko Hanaoka, Daisuke Shintani, Sho Sato, Masanori Yasuda, Akira Yabuno
    JAPANESE JOURNAL OF GYNECOLOGIC AND OBSTETRIC ENDOSCOPY.2022; 38(1): 158.     CrossRef
  • Primary retroperitoneal PEComa: an incidental finding
    Bárbara Monteiro Marinho, António Gâmboa Canha, Donzília Sousa Silva, José Davide Pinto Silva
    BMJ Case Reports.2022; 15(11): e250466.     CrossRef
  • Imaging Findings of Thoracic Lymphatic Abnormalities
    Jingshuo (Derek) Sun, Thomas Shum, Fardad Behzadi, Mark M. Hammer
    RadioGraphics.2022; 42(5): 1265.     CrossRef
  • Extrapulmonary uterine lymphangioleiomyomatosis (LAM) and dysfunctional uterine bleeding: the first presentation of LAM in a tuberous sclerosis complex patient
    Lucy Grant, Saliya Chipwete, San Soo Hoo, Anjali Bhatnagar
    BMJ Case Reports.2019; 12(2): e226358.     CrossRef
  • Summary of the Japanese Respiratory Society statement for the treatment of lung cancer with comorbid interstitial pneumonia
    Takashi Ogura, Nagio Takigawa, Keisuke Tomii, Kazuma Kishi, Yoshikazu Inoue, Eiki Ichihara, Sakae Homma, Kazuhisa Takahashi, Hiroaki Akamatsu, Satoshi Ikeda, Naohiko Inase, Tae Iwasawa, Yuichiro Ohe, Hiromitsu Ohta, Hiroshi Onishi, Isamu Okamoto, Kazumasa
    Respiratory Investigation.2019; 57(6): 512.     CrossRef
  • Incidental lymphangioleiomyomatosis in the lymph nodes of gynecologic surgical specimens
    Ikumi Kuno, Hiroshi Yoshida, Hanako Shimizu, Takashi Uehara, Masaya Uno, Mitsuya Ishikawa, Tomoyasu Kato
    European Journal of Obstetrics & Gynecology and Reproductive Biology.2018; 231: 93.     CrossRef
  • Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review
    Weiwei Fu, Yujun Li, Hong Li, Ping Yang, Xiaoming Xing
    Experimental and Therapeutic Medicine.2016; 12(3): 1499.     CrossRef
  • Incidental Pelvic and Para-aortic Lymph Node Lymphangioleiomyomatosis Detected During Surgical Staging of Pelvic Cancer in Women Without Symptomatic Pulmonary Lymphangioleiomyomatosis or Tuberous Sclerosis Complex
    Joseph T. Rabban, Brandie Firetag, Ankur R. Sangoi, Miriam D. Post, Charles J. Zaloudek
    American Journal of Surgical Pathology.2015; 39(8): 1015.     CrossRef
Usual Interstitial Pneumonia with Lung Cancer: Clinicopathological Analysis of 43 Cases
Dae Hyun Song, In Ho Choi, Sang Yun Ha, Kang Min Han, Jae Jun Lee, Min Eui Hong, Kyeongman Jeon, Man Pyo Chung, Jhingook Kim, Joungho Han
Korean J Pathol. 2014;48(1):10-16.   Published online February 25, 2014
DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.1.10
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  • 15 Crossref
AbstractAbstract PDF
Background

Previous studies have suggested an association between usual interstitial pneumonia (UIP) and lung cancer (Ca). However, clinical and histological information is not enough to determine such an association, due to the low incidence and short survival time of patients with both conditions.

Methods

We retrospectively reviewed the clinical and histological records of Ca patients with UIP between January 1999 and August 2013 at the Samsung Medical Center, Seoul, Korea. We found 43 patients who had Ca with UIP (UIP-Ca). Previously reported data of eighty-four patients with UIP-only were included as a comparison group.

Results

Smoking is related to poor prognosis in patients with UIP-Ca, and the number of patients with a high smoking index of more than 30 pack-years significantly increased in UIP-Ca patients compared with UIP-only patients. There is no significant prognostic differentiation between UIP-Ca patients and UIP-only patients. Microscopically, UIP-Ca patients showed characteristically heterogeneous histological patterns and degrees of differentiation. There were many foci of squamous metaplasia or dysplasia at the peripheral area of squamous cell carcinomas.

Conclusions

We report 43 cases of UIP-Ca. Our results suggest that smoking is related to cancer occurrence in UIP patients and poor prognosis in UIP-Ca patients.

Citations

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    European Journal of Cardio-Thoracic Surgery.2016; 50(3): 428.     CrossRef
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    Diane C. Strollo, Teri J. Franks, Jeffrey R. Galvin
    Seminars in Roentgenology.2015; 50(1): 8.     CrossRef
  • Do Chest Expansion Exercises Aid Re‐shaping the Diaphragm Within the First 72 Hours Following Lung Transplantation in a Usual Interstitial Pneumonia Patient?
    Massimiliano Polastri, Erika Venturini, Saverio Pastore, Andrea Dell'Amore
    Physiotherapy Research International.2015; 20(3): 191.     CrossRef
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    Marie-Louise M. Coussa-Koniski, Pia A. Maalouf, Nehme E. Raad, Noha A. Bejjani
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  • The Ratio KL-6 to SLX in Serum for Prediction of the Occurrence of Drug-Induced Interstitial Lung Disease in Lung Cancer Patients with Idiopathic Interstitial Pneumonias Receiving Chemotherapy
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    Chinese Medical Journal.2014; 127(17): 3142.     CrossRef
Cytologic Features of ALK-Positive Pulmonary Adenocarcinoma
Seung Yeon Ha, Jungsuk Ahn, Mee Sook Roh, Joungho Han, Jae Jun Lee, Boin Lee, Jun Yim
Korean J Pathol. 2013;47(3):252-257.   Published online June 25, 2013
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.3.252
  • 6,864 View
  • 29 Download
  • 9 Crossref
AbstractAbstract PDF
Background

The aim of this study was to determine the cytologic features of anaplastic lymphoma kinase (ALK) expressing pulmonary adenocarcinoma.

Methods

We analyzed the cytopathological findings of 15 cases of endobronchial ultrasound guided aspiration and a case of bronchial washing. These cases were selected based on the histomorphology of ALK-rearranged lung adenocarcinoma.

Results

Cytology showed mucinous (81.3%) and hemorrhagic (50%) backgrounds. The cells were arranged in tubulopapillary or tubulocribriform patterns (93.8%), and clusters (56.3%) admixed with signet ring cell features (87.5%). The tumor cells were monotonous and uniform with vesicular nuclei and a small nucleolus.

Conclusions

The characteristic findings were sheets showing a tubulopapillary or tubulocribriform appearance, with vesicular nuclei and a bland chromatin pattern (p<0.001). Scattered signet ring cells were helpful in suggesting ALK-positive adenocarcinoma (p<0.001).

Citations

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    Pathology.2016; 48(4): 325.     CrossRef
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Prognostic Significance of BCL9 Expression in Hepatocellular Carcinoma
Jiyeon Hyeon, Soomin Ahn, Jae Jun Lee, Dae Hyun Song, Cheol-Keun Park
Korean J Pathol. 2013;47(2):130-136.   Published online April 24, 2013
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.2.130
  • 7,367 View
  • 53 Download
  • 18 Crossref
AbstractAbstract PDF
Background

BCL9 enhances β-catenin-mediated transcriptional activity regardless of the mutational status of the Wnt signaling components and increases the cell proliferation, migration, invasion, and metastatic potential of tumor cells. The goal of this study was to elucidate the prognostic significance of BCL9 protein expression in hepatocellular carcinoma (HCC) patients.

Methods

We evaluated BCL9 protein expression by immunohistochemistry in tumor tissue from 288 primary HCC patients who underwent curative hepatectomy. The impact of BCL9 expression on the survival of the patients was analyzed. The median follow-up period was 97.1 months.

Results

Nuclear BCL9 protein expression was observed in 74 (25.7%) of the 288 HCCs. BCL9 expression was significantly associated with younger age (p=0.038), higher Edmondson grade (p=0.001), microvascular invasion (p=0.013), and intrahepatic metastasis (p=0.017). Based on univariate analyses, BCL9 expression showed an unfavorable influence on both disease-free survival (DFS, p=0.012) and disease-specific survival (DSS, p=0.032). Multivariate analyses revealed that higher Barcelona Clinic Liver Cancer stage was an independent predictor of both shorter DFS (p<0.001) and shorter DSS (p<0.001). BCL9 expression tended to be an independent predictor of shorter DFS (p=0.078).

Conclusions

BCL9 protein expression might be a marker of shorter DFS in HCC patients after curative hepatectomy.

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Methylation and Immunoexpression of p16INK4a Tumor Suppressor Gene in Primary Breast Cancer Tissue and Their Quantitative p16INK4a Hypermethylation in Plasma by Real-Time PCR
Jae Jun Lee, Eunkyung Ko, Junhun Cho, Ha Young Park, Jeong Eon Lee, Seok Jin Nam, Duk-Hwan Kim, Eun Yoon Cho
Korean J Pathol. 2012;46(6):554-561.   Published online December 26, 2012
DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.6.554
  • 6,983 View
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  • 17 Crossref
AbstractAbstract PDF
Background

The p16INK4a gene methylation has been reported to be a major tumorigenic mechanism.

Methods

We evaluated the methylation status of the p16INK4a genes in 231 invasive breast cancer and 90 intraductal carcinoma specimens using a methylation-specific polymerase chain reaction and p16 protein expression using immunohistochemistry. The quantity of cell-free methylated p16INK4a DNA in the plasma samples of 200 patients with invasive breast cancer was also examined using a fluorescence-based real-time polymerase chain reaction assay.

Results

The frequencies of p16INK4a methylation in invasive and intraductal tumors were 52.8% (122/231) and 57.8% (52/90), respectively. The p16 protein was overexpressed in 145 of the 231 invasive carcinomas (62.8%) and 63 of the 90 intraductal carcinomas (70%). High p16 expression in invasive carcinomas correlated significantly with a high histologic grade, a negative estrogen receptor and progesterone receptor status, p53 immunoreactivity and high Ki-67 expression with immunohistochemistry. In addition, the methylation index of p16INK4a was significantly higher in the cancer patients than the normal controls (p<0.001).

Conclusions

High p16 immunoreactivity correlated with a loss of differentiation in breast carcinomas and high frequency of p16INK4a promoter methylation in both invasive and intraductal carcinomas, suggesting it may be involved in the pathogenesis of breast cancer.

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Clinicopathological Analysis of 21 Thymic Neuroendocrine Tumors
Soomin Ahn, Jae Jun Lee, Sang Yun Ha, Chang Ohk Sung, Jhingook Kim, Joungho Han
Korean J Pathol. 2012;46(3):221-225.   Published online June 22, 2012
DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.3.221
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AbstractAbstract PDF
Background

Thymic neuroendocrine carcinomas (NECs) are uncommon, for which there is no established information available because of a limited number of epidemiological study in Asia.

Methods

We reviewed 21 cases of surgically resected thymic NECs, and evaluated their pathological and clinical features.

Results

It showed male predominance (male/female ratio, 15/6) with wide age range from 20 to 72 years (mean age, 49 years). All 21 cases were divided into two types according to the World Health Organization criteria: atypical carcinoid (n=18) and large cell NEC (n=3). Three cases of atypical carcinoid (AC) were associated with ectopic Cushing's syndrome. All the patients (3/3) with large cell NEC (3/3) and 16.7% (3/18) of those with AC died of tumor progression. Common sites of metastasis included lung, lymph node, brain, lumbar spine, mediastinum, bone, and liver.

Conclusions

In conclusion, thymic neuroendocrine tumors carry a poor prognosis. Regarding the tumor classification, our results showed that a vast majority of carcinoids in the thymus correspond to ACs. In addition, our results also indicate that typical carcinoid is a very rare entity. Some cases of AC exhibited a large size, solid pattern and they showed aggressive clinical behavior, which highlights the spectrum of histologic appearances of thymic NECs.

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Case Report
Localized Primary Thymic Amyloidosis Presenting as a Mediastinal Mass: A Case Report.
Sang Yun Ha, Jae Jun Lee, Heejung Park, Joungho Han, Hong Kwan Kim, Kyung Soo Lee
Korean J Pathol. 2011;45:S41-S44.
DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S41
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AbstractAbstract PDF
We herein describe a case of a 55-year-old healthy woman with localized primary thymic amyloidosis presented as a mediastinal mass, found incidentally by chest radiography. Computed tomography revealed a 4.1 cm soft tissue lesion with nodular calcification in the left anterior mediastinum. The resected specimen was a well-defined lobulating mass with calcification. Microscopically, the mass was consisted of amorphous eosinophilc hyalinized substances involving the thymus and intrathymic lymph nodes. These eosinophilic substances showed apple-green bi-refringence under polarized light after staining with Congo red. In immunohistochemical study, they were positive for kappa and lambda light chains and negative for amyloid A. There was no evidence of systemic amyloidosis in clinical investigations. A final diagnosis of localized primary thymic amyloidosis was made.

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Original Article
Mucoepidermoid Carcinoma of Tracheobronchial Tree: Clinicopathological Study of 31 Cases.
Sang Yun Ha, Joungho Han, Jae Jun Lee, Young Eun Kim, Yoon La Choi, Hong Kwan Kim
Korean J Pathol. 2011;45(2):175-181.
DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.2.175
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AbstractAbstract PDF
BACKGROUND
All aspects of mucoepidermoid carcinoma (MEC) of the lung including histologic grading, clinical behavior and its differentiation from adenosquamous cell carcinoma are still not fully understood.
METHODS
We reviewed the hematoxylin-eosin stained slides and medical records of 31 cases of MEC of the lungs. The cases were classified as low and high grade according to the quantitative grading system formulated for MEC. High grade tumors were tested for an epidermal growth factor receptor (EGFR) mutation.
RESULTS
Twenty eight cases were classified as low grade and 3 cases as high grade. Histologically, lower glandular component, cellular atypia, necrosis, mitoses >4/10 high power fields, and endolymphatic tumor emboli were typical characteristics of a high grade tumor. Although some tumors showed histologic features mimicking high grade tumors, they were classified as low grade tumors according to this quantitative grading system. Low grade tumors showed no recurrence or metastasis. However, among three patients with a high grade tumor, two had distant metastases and one died of disease. Additionally, an EGFR mutation was not detected.
CONCLUSIONS
A high grade MEC was consistently different from a low grade tumor with regard to malignant histologic features and poor prognosis. Therefore, correct histologic grading is important in predicting the prognosis to avoid unnecessary treatment.

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