대한흉부영상의학회 Weekly Chest Cases

Case No : 972 Date 2016-06-13

Courtesy of Seungho Lee, Min Jae Cha / Chung-Ang university hospital
Age/Sex 21 / M
Chief ComplaintCough and sputum for 4 months. Weight loss and dyspnea.

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2014-03-07

Diagnosis With Brief Discussion

Diagnosis: Anaplastic Large Cell Lymphoma (Originating from the lung)
Radiologic Findings: (A, B) Serial chest radiographs obtained on 2014-03-07 and 2016-04-24 demonstrate interval development of a large mass with necrotic portion involving right hemithorax, associated with a small right pleural effusion.
(C~F) Axial and coronal view images of contrast-enhanced Chest CT demonstrate an approximately 16-cm-sized heterogeneous attenuation mass in right hemithorax and anterior mediastinum. The mass contains mixed solid and cystic components with air in it. Lung parenchymal invasion of the right upper and middle lobes is noted. Invasion to the superior vena cava and adjacent pericardium is also suspected. Another 1.5-cm-sized enhancing nodule in the left paraaortic area is noted separately.
Brief Review: Anaplastic large cell lymphoma (ALCL) is a distinct sub-entity of non-Hodgkin’s lymphoma (NHL) which was positive for Ki-1. This disease is characterized by the proliferation of pleomorphic large neoplastic lymphoid cells, which strongly express the CD30 antigen (Ki-1 antigen), usually growing in a cohesive pattern and preferentially spreading in the lymph node sinuses. This disease usually expresses antigens of T or B cell lineage although a few cases have no lymphoid antigens or both B and T cell lineages. A high fraction of cases which express translocation involving anaplastic lymphoma kinase (ALK) result more favorable clinical outcome than ALK negative cases. This patient was proven to be ALCL which had both B and T cell lineages and no translocation of ALK.
ALCL that accounts for 1.5-3% of all NHL in Asia and Europe has distinct subset of primary cutaneous type and systemic type. Systemic ALCL is commonly diagnosed in young male patients. They usually present with generalized extensive lymphadenopathy, but the disease may also involve extranodal organs such as bone, soft tissue, skin, lung, pleura and GI tract. Most patients present with disseminated stage disease.
Primary ALCL originating from the lung is extremely rare. Its nonspecific radiologic and clinical features make it challenging to diagnose. Radiologic features of ALCL of the lung, includes a solitary mass, multiple pulmonary nodules, bronchovascular bundle and interlobular septal thickening, and endobronchial mass causing atelectasis. Mediastinal and hilar lymphadenopathy are often present. Pleural or pericardial effusion is also reported to be frequently associated. Definite diagnosis comes from histopathologic evaluation. Once the ALCL of the lung is confirmed, systemic survey is necessary to identify extra-thoracic organ involvement, such as mesentery, peritoneum, LNs and GI tract.
References: 1. Tilly H, Gaulard P, Lepage E, et al. Primary anaplastic large-cell lymphoma in adults: clinical presentation, immunophenotype, and outcome. Blood 1997; 90:3727.
2. Lee HJ, Im JG, Goo JM, et al. Peripheral T-Cell Lymphoma: Spectrum of Imaging Findings with Clinical and Pathologic Features. RadioGraphics 2003; 23:7–28
3. Swerdlow SH, Campo E, Harris NL, et al. (Eds). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.
4. Kanthan R, Senger J-L, Miller G, et al. Isolated pediatric endobronchial primary anaplastic large cell lymphoma Journal of pediatric surgery case reports 2014.01.004
5. Hu Y, Liu P, Gao L et al. Diffuse pulmonary infiltration by anaplastic lymphoma kinase negative anaplastic large cell lymphoma: A case report and literature review. Respiratory medicine CME 2009.09.024
6. Zhao Q, Liu Y, Chen H et al. Successful Chemo-Radiotherapy for Primary Anaplastic Large Cell Lymphoma of the Lung: A Case Report and Literature Review. Am J Case Rep. 2016; 17: 70–75.
Keywords: Lung, Lymphproliferative disorder,

No. of Applicants : 91

▶ Correct Answer : 7/91, 7.7%

- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- DRRPGMC KANGRA AT TANDA , India Dinesh Sood
- 紐吏蹂 , Korea (South) KiEon Kwon
- Kyungpook national university hospital, Korea , Korea (South) Sung Jun Moon
- Avrasya Hospital , Turkey Murat Ulusoy
- Private sector , Greece Vasilios Tzilas

▶ Correct Answer as Differential Diagnosis : 20/91, 22.0%

- Hyogo Prefectural Amagasaki General Medical Center , Japan Tomoaki Otani
- Otsu Municipal Hospital , Japan Noriatsu Ichiba
- Shiga University of Medical Science , Japan Akitoshi Inoue
- University of Tsukuba Hospital , Japan Shioto Oda
- Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
- Asan Medical Center , Korea (South) Hyung Jung Koo
- Amagasaki General Medical Center , Japan Genki Fukumoto
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Gifu University Hospital , Japan Yo Kaneko
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- Okayama University Hospital , Japan Toshiyuki Komaki
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Seoul National University Bundang Hospital , Korea (South) Jee Hye Hur
- CHU Poitiers , France CHAN paul
- Aerospace medical group, Air Force Education and Training Command , Korea (South) Donghwan Kim
- Fortis hospital , Mohali , India Shaleen Rana
- Kizawa Memorial Hospital , Japan Shoji Okuda
- The University of Tokyo Hospital , Japan Kaoru Sumida
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 972 Date 2016-06-13

Discussion

No. of Applicants : 91