Case Presentation: An Atypical Mediastinal Mass

Robert Yeh
Gillian Gillian Lieberman,
Lieberman, MD
Case Presentation:
An Atypical Mediastinal
January 2002
Mass
Robert Yeh, Yeh,
Harvard Medical School Year III
Gillian Lieberman, MD

Robert Yeh
Gillian Lieberman, MD
History
20 year old asymptomatic African-American
African American
female seeking left breast reconstruction.
History includes:
• Left chest wall cyst removed as an infant
• Left arm “oozing sac” removed at age 10
• Sickle cell trait
2

Robert Yeh
Gillian Lieberman, MD
Our Patient:
Pre-Op Pre Op Workup Chest X-Ray X Ray
From PACS, BIDMC
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Robert Yeh
Gillian Lieberman, MD
Trachea deviated to right
Unilateral breast shadow
Our Patient
Rib
From PACS, BIDMC
Mass
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Robert Yeh
Gillian Lieberman, MD
Obliteration of
retrosternal space
Our Patient
Position in anterior mediastinum narrows differential diagnosis
From PACS, BIDMC
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Robert Yeh
Gillian Lieberman, MD
The Mediastinal
Anterior Mediastinum
• Anterior to the
pericardium and
brachiocephalic vessels
behind the sternum
• Contains fat, thymic
remnants, internal
mammary vessels, and
lymph nodes
Compartments
From Moore, EH. Chest Radiology.
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Robert Yeh
Gillian Lieberman, MD
Anterior Mediastinal
Thymoma
Teratoma
Terrible Lymphoma
Ectopic Thyroid
“The The Four Ts”
Others are rare:
Thymic Carcinoma
Thymic Carcinoid
Thymolipoma
Seminoma
Lymphangioma
Parathyroid Adenoma
Metastatic Disease
Masses
One-half of all mediastinal masses are in the anterior
mediastinum
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Robert Yeh
Gillian Lieberman, MD
Characteristics
• Most common anterior mediastinal
tumor in adults (20%)
• Usually age > 40
• Associated with myasthenia gravis
and pure red cell aplasia
Plain Film
• Well-defined, Well defined, rounded, or lobulated
on one side of the midline
• Visible calcifications rare and
usually small, curvilinear, or
punctate
Thymoma
From PACS,
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Robert Yeh
Gillian Lieberman, MD
Characteristics
• 10% of all anterior mediastinal
tumors, most common in infants
• May rarely contain malignant foci
• Often cystic within the mediastinum
Plain Film
• Usually protrude to one side of
midline and can reach large sizes
• 26% exhibit calcification and may
display recognizable bone or teeth
• Often lower in mediastinum
Teratoma
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From http://www.med.univ-rennes1.fr/cerf/iconocerf/P/Dossier_MEDI-003233_-P_0252.html

Robert Yeh
Gillian Lieberman, MD
Characteristics
• 10-20% 10 20% of anterior mediastinal
tumors
• Hodgkin’s Lymphoma is most
common
• Nodular sclerosing form favors
anterior mediastinum
• May present with fever, night
sweats, and/or weight loss
Plain Film
• Discrete lobulated mass
• Often bilateral asymmetric nodal
disease with contiguous spread
along lymph node chains
• Calcifications rare before therapy
Lymphoma
From http://info.med.yale.edu/intmed/cardio/imaging/cases/lymphoma/
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Robert Yeh
Gillian Lieberman, MD
Characteristics
• 10% of mediastinal masses
• Rarely malignant
• Most commonly in asymptomatic
women with a palpable cervical
goiter
Plain Film
• Encapsultated,
Encapsultated,
lobulated and
heterogeneous
• Continuity between the cervical
and mediastinal components
• Punctate or course calcifications
common
Ectopic Thyroid
From www.thyroidimaging.com/ rx_goz1.jpg
All 4 Ts are often asymptomatic and found incidentally
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Robert Yeh
Gillian Lieberman, MD
Utility of Plain Film
Ahn et al. 1996 found 36% accuracy of first
diagnosis of anterior mediastinal masses on
plain film by two separate radiologists
“I I suspect the presence of a superior mediastinal
mass in a 20 year old patient. This could in part
relate to thymic soft tissue. However, further
evaluation with chest CT is recommended to
exclude the presence of a mass.”
- Radiologist report
From Ahn, JM, et al. J Thorac Imaging 1996 Fall; 11(4)265-71
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Robert Yeh
Gillian Lieberman, MD
Our Patient:
Mass
Chest CT
• Soft tissue attenuation
• Well defined border
• Heterogenous
• Envelops great vessels
Aortic Arch
Tracheal bifurcation
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient:
Chest CT
Pulmonary Trunk
Aorta
Mass postero-medial
postero medial
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient:
CT in Abdomen
Abdominal sections
• Numerous low
attenuation cystic
structures in spleen
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient:
Lung Windows
• Significantly reduced
left lung volume
despite patient being
asymptomatic
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
CT report
“IMPRESSION:
IMPRESSION:
Extensive mediastinal soft tissue mass. The
appearance is most suggestive of a neoplastic
process. Given the patient's age and associated
findings of multiple splenic lesions, the most
likely
consideration would be that of lymphoma. Other
considerations include infection, such as TB
and
fungal disease.” disease.
Has the differential diagnosis changed?
-Radiologist Radiologist Report
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Robert Yeh
Gillian Lieberman, MD
Our Patient:
CT-guided CT guided Needle Biopsy
Report:
• “A 19-gauge coaxial core biopsy
needle was inserted into the left
anterior mediastinal mass under
CT guidance and its position
within the mass confirmed.”
Cytology:
• “Predominantly blood, scattered
stromal cells, lipid-laden
histiocytes, mesothelial cells and
lymphocytes present.”
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Robert Yeh
Gillian Lieberman, MD
Our Patient:
MRI
“Multiple small cystic areas
with fluid-filled levels….this
mass is also intimately
associated with the aortic arch
and the pulmonary trunk”
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient: MRI
“It extends superiorly to the left lower neck, laterally to the left axilla.”
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From From PACS, PACS, BIDMC BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient
“The spleen also demonstrates multiple cystic areas. There is splenomegaly.”
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient
“The The aforementioned mass is consistent with lymphatic
malformation, namely cystic lymphangioma”
lymphangioma
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Our Patient
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From PACS, BIDMC

Robert Yeh
Gillian Lieberman, MD
Lymphangioma
• Histologically benign proliferation of interconnecting
lymphatic vessels and sacs that may grow in an infiltrative
fashion
• Controversial etiology: hamartoma vs. neoplasm vs.
developmental lesion
• Fifty percent are present at birth and 90% are discovered by 2
years of age
From Requena, L and O Sangueza. Journal of the American Academy of Dermatology. 37:6. December 1997
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Robert Yeh
Gillian Lieberman, MD
Lymphangioma
• 0.7-4.5% 0.7 4.5% of mediastinal tumors
• Ninety-five Ninety five percent involve the
neck or axilla
• Rarely, a generalized
lymphangiomatosis with
extensive multifocal
involvement of multiple organ
systems can occur
(cont.)
From www.edmondsmd.com/ neck_lymphangioma.jpg
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Robert Yeh
Gillian Lieberman, MD
Lymphangioma
Categorized into three types
1) simple lymphangioma: lymphangioma:
formed
by lymphatic capillaries
2) cavernous lymphangioma:
lymphangioma:
formed by bigger lymphatic
vessels with a fibrous adventitia
3) cystic lymphangioma: lymphangioma:
also
called cystic hygroma, hygroma,
formed
by multiple cysts ranging from a
few millimeters to several
centimeters in size.
Complications include chylothorax
and compression of airway, great
vessels
(cont.)
From www.ijri.org/archives/19990904/case_pg187.htm
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Robert Yeh
Gillian Lieberman, MD
Splenic
• Extremely rare condition,
but approximately 100
cases have been reported
since 1885
• Associated with
consumptive
coagulopathy,
coagulopathy,
thrombocytopenia and
portal hypertension
Lymphangiomatosis
From PACS, BIDMC
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Robert Yeh
Gillian Lieberman, MD
Patient Follow-Up Follow Up
• Referred to plastic surgeon for breast
reconstruction
• Subsequently referred to thoracic surgeon
who deferred surgery due to extensive
nature of disease
• No further records
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Robert Yeh
Gillian Lieberman, MD
References
• Ahn JM, Lee KS, Goo JM, Song KS, Kim SJ, Im JG. Predicting the histology of anterior
mediastinal masses: comparison of chest radiography and CT. Journal of Thoracic
Imaging 1996; 11(4): 265-71.
• Brown LR, Reiman HM, Rosenow EC, Glovickzki PM, Divertie MB. Intrathoracic
lymphangioma. Mayo Clinical Proceedings 1986; 61: 882-892.
• Cohn WE. Anterior mediastinal mass lesions. UpToDate, Inc., 2001
• Moore, EH. Chest Radiology. Http://alice.ucdavis.edu/IMD/420C/syllabus/Radio2.htm
• Morgenstern L, Bello JM, Fisher BL, Verham RP. The clinical spectrum of
lymphangiomas and lymphangiomatosis of the spleen. The American Surgeon 1992: 58:
599-604.
• Shaffer K, Rosado-de-Christenson ML, Patz EF, Young S, Farver CF. Thoracic
lymphangioma in adults: CT and MR imaging features. AJR 1994; 162: 283-289.
• Strollo DC, Rosado de Christenson ML, Jett JR. Primary mediastinal tumors, part 1:
tumors of the anterior mediastinum. Chest 1997; 112(2): 511-22.
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Robert Yeh
Gillian Lieberman, MD
Acknowledgements
• Daniel Saurborn, Saurborn,
MD
• Gillian Lieberman, MD
• Pamela Lepkowski
• Larry Barbaras and Cara Lyn D’amour
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