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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
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The Great Pretender
The Great Pretender
Unicentric Castleman Disease Mimicking Paraganglioma in a Young Woman
Unicentric Castleman Disease Mimicking Paraganglioma in a Young Woman
Castleman's disease is named after Dr. Benjamin Castleman (1906–1982), an eminent American pathologist who served as the Chief of Pathology at Massachusetts General Hospital (MGH). He first identified the condition in 1954 and published a definitive series of 13 cases in the journal Cancer in 1956
Contributed by : Gopal Kurwade
Contributed by : Gopal Kurwade
Introduction :
Introduction :
Castleman disease (CD) is an uncommon benign B-cell lymphoproliferative disorder, also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia. It may involve multiple anatomical regions ; but most commonly presents as a solitary mediastinal mass. CD is classified into unicentric (UCD) and multicentric (MCD) forms, with the hyaline-vascular subtype accounting for the majority of unicentric cases.
Castleman disease (CD) is an uncommon benign B-cell lymphoproliferative disorder, also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia. It may involve multiple anatomical regions ; but most commonly presents as a solitary mediastinal mass. CD is classified into unicentric (UCD) and multicentric (MCD) forms, with the hyaline-vascular subtype accounting for the majority of unicentric cases.
UCD typically presents as an asymptomatic solitary mediastinal or cervical mass in young adults and is often discovered incidentally. Radiologically, the hyaline-vascular subtype characteristically demonstrates intense homogeneous post-contrast enhancement due to its highly vascular stroma — a feature that may closely mimic paraganglioma, schwannoma, and other hypervascular neck masses.
UCD typically presents as an asymptomatic solitary mediastinal or cervical mass in young adults and is often discovered incidentally. Radiologically, the hyaline-vascular subtype characteristically demonstrates intense homogeneous post-contrast enhancement due to its highly vascular stroma — a feature that may closely mimic paraganglioma, schwannoma, and other hypervascular neck masses.
As imaging findings are not pathognomonic, histopathological examination with immunohistochemistry remains the gold standard for definitive diagnosis.
As imaging findings are not pathognomonic, histopathological examination with immunohistochemistry remains the gold standard for definitive diagnosis.
Case presentation :
Case presentation :
A 23-year-old woman presented with a gradually progressive swelling on the left side of the neck. There was no history of pain, fever, weight loss, night sweats, hypertension, palpitations, or episodic headache. No prior history of malignancy or immunosuppression was present.
A 23-year-old woman presented with a gradually progressive swelling on the left side of the neck. There was no history of pain, fever, weight loss, night sweats, hypertension, palpitations, or episodic headache. No prior history of malignancy or immunosuppression was present.
On clinical examination, a mobile painless 5 × 5 cm mass was palpable in the left supraclavicular region (Figure 1). No hepatosplenomegaly or generalized lymphadenopathy was identified.
On clinical examination, a mobile painless 5 × 5 cm mass was palpable in the left supraclavicular region (Figure 1). No hepatosplenomegaly or generalized lymphadenopathy was identified.
Fig 1
The swelling on the left side of the neck
A frontal radiograph of the cervical thoracic region was performed.
A frontal radiograph of the cervical thoracic region was performed.
Fig 2
The frontal radiograph of the cervicothoracic region shows a well-defined homogeneous soft tissue shadow across the left Cervicothoracic region.. The superior margin of the lesion is obscured above the clavicle suggestive of an anterior mediastinal lesion extending into the neck. Mild rightward tracheal deviation is also seen.
Ultrasonography of the neck demonstrated a well-defined lobulated hypoechoic lesion in the left carotid space extending inferiorly into the superior mediastinum, causing medial displacement of the left common carotid artery and compression of the left internal jugular vein (Figures 3a and 3b). Colour Doppler imaging demonstrated marked vascularity in the lesion with dense arborising vascular channels (Figure 3c). Multiple enlarged left cervical lymph nodes with normal fat in the hilum with cortical thickening were also seen.
Ultrasonography of the neck demonstrated a well-defined lobulated hypoechoic lesion in the left carotid space extending inferiorly into the superior mediastinum, causing medial displacement of the left common carotid artery and compression of the left internal jugular vein (Figures 3a and 3b). Colour Doppler imaging demonstrated marked vascularity in the lesion with dense arborising vascular channels (Figure 3c). Multiple enlarged left cervical lymph nodes with normal fat in the hilum with cortical thickening were also seen.
Figure 3
(a, b) Ultrasonography of the neck shows a well-defined lobulated hypoechoic lesion in the left carotid space extending into the superior mediastinum (white arrows), causing medial displacement of the left common carotid artery and compression of the left internal jugular vein.
(c) Colour Doppler image demonstrating marked internal vascularity with dense arborising vascular channels within the lesion (white arrowheads).
Contrast-enhanced CT of the neck demonstrated a well-defined intensely enhancing lobulated soft tissue lesion measuring 7.3 × 5 × 4.2 cm in the left infrahyoid neck, extending into the superior mediastinum (Figures 4a and 4b). The lesion compresses the left internal jugular, subclavian, and brachiocephalic veins. Mild rightward displacement of the trachea and esophagus is seen. No calcification or necrosis is seen.
Contrast-enhanced CT of the neck demonstrated a well-defined intensely enhancing lobulated soft tissue lesion measuring 7.3 × 5 × 4.2 cm in the left infrahyoid neck, extending into the superior mediastinum (Figures 4a and 4b). The lesion compresses the left internal jugular, subclavian, and brachiocephalic veins. Mild rightward displacement of the trachea and esophagus is seen. No calcification or necrosis is seen.
Figure 4
(a, b) Contrast-enhanced CT images of the neck shows a well-defined intensely enhancing lobulated soft tissue lesion in the left infrahyoid neck extending into the superior mediastinum (white arrows). The lesion causes compression of the left internal jugular and brachiocephalic veins with mild rightward displacement of the trachea (black arrowhead).
Radiological differential diagnosis:
Radiological differential diagnosis:
The differential diagnosis of a hypervascular neck mass in a young adult includes:
The differential diagnosis of a hypervascular neck mass in a young adult includes:
1. Paraganglioma — Hypervascular lesion in the carotid space; may splay CCA and IJV; can secrete catecholamine’s; SSTR avid on scintigraphy. Indistinguishable from Castleman disease on CT alone.
1. Paraganglioma — Hypervascular lesion in the carotid space; may splay CCA and IJV; can secrete catecholamine’s; SSTR avid on scintigraphy. Indistinguishable from Castleman disease on CT alone.
2. Castleman Disease (Hyaline-Vascular type) — Intense homogeneous enhancement; no catecholamine secretion; defined by histopathology showing atretic follicles, twinned germinal centres, and fibrotic high endothelial venules.
2. Castleman Disease (Hyaline-Vascular type) — Intense homogeneous enhancement; no catecholamine secretion; defined by histopathology showing atretic follicles, twinned germinal centres, and fibrotic high endothelial venules.
3. Schwannoma — Well-defined; heterogeneous enhancement; target sign on MRI; neural foraminal widening; Antoni A/B areas on histology.
3. Schwannoma — Well-defined; heterogeneous enhancement; target sign on MRI; neural foraminal widening; Antoni A/B areas on histology.
4. Lymphoma — Enlarged lymph node conglomerate; less intense enhancement; systemic B symptoms; PET-avid.
4. Lymphoma — Enlarged lymph node conglomerate; less intense enhancement; systemic B symptoms; PET-avid.
5. Reactive lymphadenopathy — Normal hilar architecture; less avid enhancement; clinical context of infection or inflammation.
5. Reactive lymphadenopathy — Normal hilar architecture; less avid enhancement; clinical context of infection or inflammation.
Radiological diagnosis
Radiological diagnosis
Left infrahyoid Paraganglioma
Left infrahyoid Paraganglioma
Pathological diagnosis:
Pathological diagnosis:
Hyaline-vascular subtype of unicentric Castleman disease
Hyaline-vascular subtype of unicentric Castleman disease
Management:
Management:
In view of the lesion's size, superior mediastinal extension, and mass effect on adjacent vascular structures, the patient underwent multidisciplinary evaluation followed by surgical excision.
In view of the lesion's size, superior mediastinal extension, and mass effect on adjacent vascular structures, the patient underwent multidisciplinary evaluation followed by surgical excision.
Intra-operatively, a 10 × 6 cm mass was identified involving the left phrenic nerve, thoracic duct, left internal jugular vein, and left lateral thoracic artery, with extension below the clavicle into the superior mediastinum.
Intra-operatively, a 10 × 6 cm mass was identified involving the left phrenic nerve, thoracic duct, left internal jugular vein, and left lateral thoracic artery, with extension below the clavicle into the superior mediastinum.
The gross examination revealed a well-circumscribed encapsulated nodal mass measuring 7.9 × 3.8 × 2.9 cm with homogeneous gray-white cut surface and no areas of hemorrhage or necrosis (Figure 5).
The gross examination revealed a well-circumscribed encapsulated nodal mass measuring 7.9 × 3.8 × 2.9 cm with homogeneous gray-white cut surface and no areas of hemorrhage or necrosis (Figure 5).
The postoperative course was uneventful; the patient was discharged in satisfactory condition.
The postoperative course was uneventful; the patient was discharged in satisfactory condition.
Figure 5
Gross pathological specimen shows a well-circumscribed, encapsulated nodal mass (white arrow) with homogeneous gray-white cut surface and no areas of hemorrhage or necrosis.
Discussion
Discussion
Castleman Disease is a rare non-clonal lymphoproliferative disorder first described by Benjamin Castleman in 1956. The hyaline-vascular subtype accounts for the majority of unicentric cases and is characterized by marked vascular proliferation and hyalinized follicles on histopathology [1].
Castleman Disease is a rare non-clonal lymphoproliferative disorder first described by Benjamin Castleman in 1956. The hyaline-vascular subtype accounts for the majority of unicentric cases and is characterized by marked vascular proliferation and hyalinized follicles on histopathology [1].
Clinically, unicentric Castleman disease commonly presents as a solitary asymptomatic mediastinal or cervical mass in young adults [2]. The absence of systemic symptoms and near-normal laboratory parameters in the present case were consistent with the hyaline-vascular subtype.
Clinically, unicentric Castleman disease commonly presents as a solitary asymptomatic mediastinal or cervical mass in young adults [2]. The absence of systemic symptoms and near-normal laboratory parameters in the present case were consistent with the hyaline-vascular subtype.
The intense homogeneous enhancement on CT and marked vascularity on Doppler ultrasound reflect the hypervascular nature of the lesion [3]. These imaging findings closely mimic paraganglioma, particularly when the lesion is located within the carotid space causing displacement of adjacent vessels. Although MRI may help differentiate the two entities by demonstrating the classic “salt-and-pepper” appearance of paraganglioma, CT-based differentiation alone may be difficult [3,4].
The intense homogeneous enhancement on CT and marked vascularity on Doppler ultrasound reflect the hypervascular nature of the lesion [3]. These imaging findings closely mimic paraganglioma, particularly when the lesion is located within the carotid space causing displacement of adjacent vessels. Although MRI may help differentiate the two entities by demonstrating the classic “salt-and-pepper” appearance of paraganglioma, CT-based differentiation alone may be difficult [3,4].
In the present case, the absence of catecholamine-related symptoms and the initial biopsy findings of reactive lymphadenopathy were important clues against paraganglioma. However, definitive diagnosis was established only after surgical excision and histopathological examination with immunohistochemistry.
In the present case, the absence of catecholamine-related symptoms and the initial biopsy findings of reactive lymphadenopathy were important clues against paraganglioma. However, definitive diagnosis was established only after surgical excision and histopathological examination with immunohistochemistry.
Complete surgical excision is curative in unicentric Castleman disease and is associated with excellent long-term prognosis [4]. The complex vascular and mediastinal relations in the present case highlight the importance of pre-operative cross-sectional imaging for surgical planning.
Complete surgical excision is curative in unicentric Castleman disease and is associated with excellent long-term prognosis [4]. The complex vascular and mediastinal relations in the present case highlight the importance of pre-operative cross-sectional imaging for surgical planning.
Conclusion:
Conclusion:
Unicentric hyaline-vascular Castleman Disease should be considered in the differential diagnosis of hypervascular neck or superior mediastinal masses in young adults, as imaging findings may closely mimic paraganglioma. Histopathological examination remains essential for definitive diagnosis, and complete surgical excision is usually curative.
Unicentric hyaline-vascular Castleman Disease should be considered in the differential diagnosis of hypervascular neck or superior mediastinal masses in young adults, as imaging findings may closely mimic paraganglioma. Histopathological examination remains essential for definitive diagnosis, and complete surgical excision is usually curative.
References:
References:
1. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956;9(4):822–830.
1. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956;9(4):822–830.
2. Fajgenbaum DC, Uldrick TS, Bhatt A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646–1657.
2. Fajgenbaum DC, Uldrick TS, Bhatt A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646–1657.
3. Ko SF, Hsieh MJ, Ng SH, et al. Imaging spectrum of Castleman's disease. AJR Am J Roentgenol. 2004;182(3):769–775.
3. Ko SF, Hsieh MJ, Ng SH, et al. Imaging spectrum of Castleman's disease. AJR Am J Roentgenol. 2004;182(3):769–775.
4. Bonekamp D, Horton KM, Hruban RH, Fishman EK. Castleman disease: the great mimic. Radiographics. 2011;31(6):1793–1807.
4. Bonekamp D, Horton KM, Hruban RH, Fishman EK. Castleman disease: the great mimic. Radiographics. 2011;31(6):1793–1807.
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