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Weekly Chest CasesArchive of Old Cases

Case No : 619 Date 2009-09-06

  • Courtesy of Woon-Jung Kwon / Ulsan University Hospital
  • Age/Sex 14 / M
  • Chief ComplaintCough, orthopnea
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7
  • Figure 8

Diagnosis With Brief Discussion

Diagnosis
Hodgkins lymphoma (nodular sclerosing type)
Radiologic Findings
A 14 years old boy presented with cough and orthopnea. At that time, CT scan showed heterogenous enhancing soft tissue mass with encasement of both main bronchi and invasion of the left chest wall in the anterior mediastinum. When the excisional biopsy was done at the left chest wall, the pathologic diagnosis was fibrosing mediastinitis without any malignant cells. After low dose steroid therapy, the mediastinal mass size has been reduced.
As time goes by, both neck swelling was newly developed. Five months later the CT scan shows heterogenous enhancing mass in both supraclavicular spaces as well as the anterior mediastinum. The neck mass displaced the carotid arteries and jugular veins. The excisional biopsy of the neck mass was performed for the definitive diagnosis.

After 5 months

After 5 months

After 5 months

Larger pleomorphic tumor cells (white arrow) with prominent nucleoli was seen in H&E stain. These tumor cells composed o

The tumor cells demonstrated strong positive immunoreaction for CD4 and CD30(Ki-1) showing cell surface staining.

Brief Review
ALCL is a distinct from of NHL that affects children and young adults predominantly. It was described first by Stein et al in 1985 when it was noted that a subset of malignant lymphomas expressed CD30 (Ki-1 antigen). Morphologically, these CD30+ lymphomas are composed of large cells with bizarre nuclei often co-expressing T-cell antigens. Prior to the recognition of CD30 staining, ALCL was classified in a variety of ways. ALCL is a T-cell NHL with a unique morphology, immunohistochemical staining pattern, cytogenetic abnormalities, and pathobiology. The unique genetic feature of ALCL is translocation involving the ALK gene located on chromosome 2p23.
ALCL is relatively uncommon, accounting for 3% of all NHLs. Systemic ALCL is predominantly a disease of childhood and adolescence, and represents 10%-15% of all childhood NHLs and 30%-40% of large cell lympomas in the pediatric population. ALCL is most common in the first three decades of life, with a marked male predominance, especially in younger patients.
Systemic ALCL presents with advanced stage (stages III-IV) disease in 65%-75% of patients. Nodal involvement is observed in 90% of systemic ALCL patients, with 40% having exclusive nodal involvement. ALCL can present with a locally invasive mediastinal mass, although less commonly than in HL. Adverse clinical prognostic factors include mediastinal involvement, visceral involvement, elevated LDH levels, and high-intermediate/high IPI scores. Relapse occur in 20%-40% of patients and can be treated effectively with salvage chemotherapy and stem cell transplant.

Note.

At first, we couldn’t extract entire mediastinal mass, we could provisionally conclude that the diagnosis of his mediastinal mass was fibrosing mediastinitis based on the excisional biopsy result of the anterior chest wall mass and the reduction of the mass with low dose steroid therapy. Therefore, initially we thought that this case is valuable for report because fibrosing mediastinitis should be added for the differential diagnosis of the anterior mediastinal mass in adolescent. During follow-up, the neck mass was newly developed, the biopsy result of the neck mass was Hodgkins lymphoma nodular sclerosing type. However, though the final diagnosis was changed to lymphoma, we could get the value from this case as follow; when the pathologic finding of the anterior mediastinal mass by partially resected or percutaneous needle biopsy is negative for malignancy, we must suggest that the lesion may be malignancy through the imaging findings.

# Correct diagnosis : Hodgkins Lymphoma
# Differential diagnosis
-- NHL
-- Germ cell tumor
-- Fibrosing mediastinitis

References
1. CT evaluation of the anterior mediastinum: spectrum of disease. Radiographics. 1994 Sep;14(5):973-90.
Lymphoma in adolescents and young adults. Semin Oncol. 2009 Oct;36(5):381-418.
Keywords
Mediastinum, Lymphproliferative disorder, Lymphoma,

No. of Applicants : 95

▶ Correct Answer as Differential Diagnosis : 77/95,  81.1%
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - McGill University Health Centre , Canada Amr Ajlan
  • - KMC,Manipal , India satish maddukuri
  • - NTUH , Taiwan Kuei-pin Chung
  • - Trakya University School of Medicine, Turkey , Turkey Ahmet POLAT
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OKUBO
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - Radiologie Centrale CHU Saint Etienne , France Romain MALEZIEUX
  • - aarthi scans , India abirami mahadevan
  • - Vitalife Clinics , Pune , India. , India Rahul Deshmukh
  • - SMC , Korea (South) Jihoon Cha
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Seoul national university hospital , Korea (South) A Jung Chu
  • - POPOVO HOSPITAL , Bulgaria VLADISLAV RUSINOV
  • - Yashoda Superspeciality hospital , India Pravin Mahadevappa
  • - hospital Sao Paulo , Brazil israel missrie
  • - IRCCS Istituto Oncologico Bari , Italy Carlo Florio
  • - partner, aditya imaging centre , India vivek patel
  • - khartoum university sudan college of post graduate , Saudi Arabia elbagir nasser
  • - Hospital M de Deus , Brazil Thiago Bento da Silva
  • - Mackay Memorial Hospital , Taiwan Sheng-Yeh Shen
  • - HOSPITAL SOTIRIA , Greece AIKATERINI DELAVERI
  • - Seoul St. Mary , Korea (South) Myeong Ahn
  • - Assam medical college , India Karunakaran M
  • - clinique de rochefort , France viviane pages
  • - Asan Medical Center , Korea (South) Hye Jeon Hwang
  • - CH Sud Rnion , Reunion jean-baptiste Noel
  • - DCA, , India Rajesh Gothi
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - Hanmaeum Hospital , Korea (South) Kwon Hyoung Kim
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Seoul Nationial University Bundang Hospital , Korea (South) Kyoung Jin Oh
  • - CHRU Lille , France Benjamin Damarey
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) YOOKYUNG KIM
  • - SMG-SNUH boramae medical center , Korea (South) Kwang Nam Jin
  • - Bundang CHA hospital , Korea (South) Jung Jin Young
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - severance hospital , Korea (South) lee jong
  • - Seoul National University Hospital , Korea (South) Hye Jin Yoo
  • - National Taiwan University Hospital, YL branch , Taiwan Chin-Chung Shu
  • - Soonchunhyang university hospital, Bucheon , Korea (South) Hyelin Kim
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - Nirman hitech diagnostic centre , India minal seth
  • - Paramount Diagnostic & Research Centre , India Mahavir Swami
  • - Yonsei University College of Medicine , Korea (South) Young Joo Suh
  • - Saga University , Japan Ryoko Egashira
  • - IRSA , France jean-luc BIGOT
  • - Hopital universitaire Lausanne , Switzerland abed kourhani
  • - Hopital du SacrCoeur , Canada Abraham Bohadana
  • - Homs National Hospital , Syria Rami Abou Zalaf
  • - Apollo Gleneagles hosp kolkata , India SALIM PARVEZ
  • - kims,narkatpally , India k bhaskar
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - CHU Grenoble , France Fabrice Bing
  • - Toyama University Hospital, Laboratory of Pathology , Japan TANAKA TOMONORI
  • - NOUR EL-ISLAM RADIOLOGY CENTER , Egypt Elsayed Mousa
  • - Changhua Christian Hospital , Taiwan Chia-Fu Tsai
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Hital SacrCoeur, Montrl , Canada Andrea Ojanguren
  • - NASA SCANS , India RAKESH BHATIA
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - CH de la Ce Basque , France Paul ARDILOUZE
  • - UZ GASTHUISBERG , Belgium Johan COOLEN
  • - PITTWATER RADIOLOGY , Australia Julie Arora
  • - Shinsegye Rad Clinic, Daegu , Korea (South) Gi Beom Kim
  • - Armed Forces Chun-Cheon Hospital , Korea (South) ChaeHun Lim
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - CHU caen , France nicolas gautier
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Kangnam Sacred Heart Hospital , Korea (South) Jaewon Kim
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