Background: Castleman’s disease is a rare lymphoproliferative disorder. It typically presents as mediastinal masses and causes a wide range of clinical symptoms. Histologically, Castleman’s disease is classified as either a hyalinic vascular or plasma cell variant. The prognosis mainly depends on the histological type and broadly varies. We herein report our sonographic findings in a patient with Castleman’s disease, including gray-scale ultrasonography, color Doppler ultrasonography, and sonoelastography ultrasonography, which have not been previously reported in the literature. These findings allowed for a preoperative diagnosis and avoidance of overly aggressive therapy.

Case presentation: A 28-year-old European female patient with unicentric Castleman’s disease of hyalinic vascular type (HV) restricted to the axilla was referred to us because of a 4-month history of a painless, solitary mass located in the left axilla. The patient’s medical history was unremarkable.

Conclusion: Castleman’s disease is a pathologic entity of unknown etiology and pathogenesis. In this case report of unicentric HV-type CD, we demonstrate that typical sonographic findings can lead to a preoperative diagnosis of Castleman’s disease. Core needle biopsy usually allows for a final diagnosis and helps to avoid unnecessary operations and overtreatment

Maden, Zerrin

Wagner, Norbert

BMC Research Notes

English

PubMed

Norbert Wagner

Zerrin Maden

Crossref

An unusual unifocal presentation of Castleman’s disease in a young woman with a detailed description of sonographic findings to reduce diagnostic uncertainty: a case report

Hochschulschriftenserver - Universität Frankfurt am Main

CASE REPORT Open AccessAn unusual unifocal presentation of Castleman’sdisease in a young woman with a detaileddescription of sonographic findings to reducediagnostic uncertainty: a case reportNorbert Wagner1* and Zerrin Maden2AbstractBackground: Castleman’s disease is a rare lymphoproliferative disorder. It typically presents as mediastinal massesand causes a wide range of clinical symptoms. Histologically, Castleman’s disease is classified as either a hyalinicvascular or plasma cell variant. The prognosis mainly depends on the histological type and broadly varies. Weherein report our sonographic findings in a patient with Castleman’s disease, including gray-scale ultrasonography,color Doppler ultrasonography, and sonoelastography ultrasonography, which have not been previously reported inthe literature. These findings allowed for a preoperative diagnosis and avoidance of overly aggressive therapy.Case presentation: A 28-year-old European female patient with unicentric Castleman’s disease of hyalinic vasculartype (HV) restricted to the axilla was referred to us because of a 4-month history of a painless, solitary mass locatedin the left axilla. The patient’s medical history was unremarkable.Conclusion: Castleman’s disease is a pathologic entity of unknown etiology and pathogenesis. In this case reportof unicentric HV-type CD, we demonstrate that typical sonographic findings can lead to a preoperative diagnosis ofCastleman’s disease. Core needle biopsy usually allows for a final diagnosis and helps to avoid unnecessaryoperations and overtreatment.Keywords: Castleman’s disease, Giant lymph node hyperplasia, Ultrasonography, Core needle biopsyBackgroundCastleman’s disease (CD), or giant lymph node hyperpla-sia, is a rare lymphoproliferative disorder that typicallypresents as mediastinal masses. It was first described byCastleman and colleagues as a localized mass of medias-tinal lymphoid follicles in 1954. Two years later, it wasdefined as a pathologic entity of unknown etiology andpathogenesis [1,2].Clinically, CD may be localized with no major symp-toms and present as a solitary mass or swelling, or itmay be a generalized, symptomatic disease with fever,weight loss, anemia, hepatosplenomegaly, and general-ized lymphadenopathy.Histologically, the disease is also classified into twoseparate subtypes: the hyalinic vascular (HV) variant(80%–90% of cases) and the plasma cell (PC) variant(10%–20% of cases). Intermediate and mixed types havealso been reported.The prognosis mainly depends on the histological typeand shows a broad variety. Treatment can range fromcurative surgery for the solitary form to the use of ste-roids, monoclonal antibodies, chemotherapy, and radio-therapy for the multicentric type [3].We herein report on a 28-year-old female patient withunicentric CD restricted to the axilla. We describe thefindings and imaging features of gray-scale ultrasonog-raphy (US), color Doppler US, sonoelastography US, andcontrast-enhanced dynamic computed tomography (CT).A pathway to a preoperative diagnosis, management ofthe disease, and the clinical course are presented. A review* Correspondence: norbertwausu@yahoo.de1Department of Obstetrics and Gynecology, Marienhospital Essen,Hospitalstrasse 24, Essen 45329, GermanyFull list of author information is available at the end of the article© 2013 Wagner and Maden; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of theCreative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use,distribution, and reproduction in any medium, provided the original work is properly cited.Wagner and Maden BMC Research Notes 2013, 6:97http://www.biomedcentral.com/1756-0500/6/97of the literature and differential diagnoses are alsopresented.Case presentationA 28-year-old European female patient was referred tous because of a 4-month history of a painless, solitarymass located in the left axilla. She had no accompanyingcomplaints, history of fatigue, night sweats, or weightloss. Her medical history was unremarkable.On routine physical examination, a solitary enlargedl y m p hn o d ew a sd e t e c t e di nt h el e f ta x i l l ai nt h ea b s e n c eo fany breast pathology. No generalized lymphadenopathy orother organomegaly was noted. Peripheral blood countsand the erythrocyte sedimentation rate were withinnormal limits. Interestingly, the levels of lymphoprolifera-tive markers such as serum soluble IL-2R, beta 2-microglobulin, and immunoglobulins were also normal;however, the C-reactive protein level was slightly increased.The lymph node in the left axilla measured 4 cm andwas mobile, nontender, and soft in consistency. USexamination of the breast and axilla was performed withan iU22 (Philips Healthcare, Bothell, WA) and ProSound7 (Aloka, Hitachi, Zug, Switzerland) using a 12-MHz lin-ear array transducer.High-frequency, high-resolution gray-scale US revealeda well-defined, uniformly hypoechoic, ovoid axillary mass,38×17×28 mm in size. The longitudinal diameter wasgreater than the transverse diameter with a longitudinal totransverse axis ratio of more than 2. A hyperechoic fattyhilum could not be detected and was totally replaced bycortical thickening. Although soft in consistency, the le-sion could only be slightly deformed by compression withthe transducer. Color Doppler flow was performed withoptimized color Doppler parameters set at a low wall filter(80–100 Hz) and low velocity scale (pulse repetition fre-quency, 1000 Hz). Color gain was adjusted dynamically tomaximize depiction of blood vessels while avoidingartifactual color noise. Bizarre and multifocal peripheralflow was detected, whereas central or central perihilar flowwas not revealed (Figure 1). A three-dimensional andmultislice imaging scan with the capability of reproducinghigh-resolution images confirmed these B-mode findings,but could not provide additional important information.Spectral Doppler analysis along the periphery of the nodeshowed both arterial and venous pulse wave patterns. Theblood flow profile of the arteries indicated a broad range inthe resistance index, pulsatility index, and peak systolic vel-ocities varying from low to high pulsatility. Thus, no fur-ther information could be drawn on these indices.Sonoelastography US confirmed the clinical examin-ation findings: the lesion was characterized by soft tissuewith some less elastic regions of higher stiffness.An US-guided fine needle biopsy with multiple pas-sages of the needle tip through the nodal cortex wasmade to sample as much of the nodal cortex as possible.Fine needle aspiration cytology (FNAC) only revealed amixed population of small and large lymphoid cells. Inparticular, prominent vascularity with hyalinized capillarieswas not detected. The FNAC results were subsequentlyreported as “negative for malignant cells,” and histo-pathologic examination of the lymph node was advised.Therefore, US-guided core needle biopsy using a 14-Gautomated gun was performed, and a diagnosis of HV-type CD was confirmed: microscopic examination revealedmany variably sized hyperplastic follicles, progressive vas-cular proliferation, and hyalinization (Figure 2).Figure 1 Color Doppler sonogram shows peripheral vascularflow within an ovoid hypoechoic axillary lymph node.Figure 2 Histopathologic specimen shows a continuum ofabnormal germinal centers (GC) with prominent CD 23-positivefollicular dendritic cells (dense black brown staining) andsubtle vascular proliferation (original magnification, ×200). MZ,mantle zone.Wagner and Maden BMC Research Notes 2013, 6:97 Page 2 of 4http://www.biomedcentral.com/1756-0500/6/97A multislice CT scan of the head, thorax, and abdomenwas subsequently performed and allowed for the exclusionof multicentric type CD. The lymph node in the left axillaon CT was described as a well-circumscribed, homoge-neous mass lesion with moderate to intense enhancementand rapid washout (Figure 3). The patient underwent openbiopsy by a surgical gynecologist, and the enlarged axillarylymph node was completely excised (Figure 4). The post-operative course was uneventful, clinical follow-upswere unremarkable, and there has been no evidence ofrecurrence.DiscussionCD is a rare, benign lymphoproliferative disorder of un-known etiology. The two main hypotheses for its develop-ment are an abnormal immune response and viralinfection. Human herpes virus 8 and interleukin 6 areregarded to be linked to the pathogenesis [4]. Microscopic-ally, as stated above, two histological subtypes are known:the HV type and PC type. Depending on the clinical pres-entation, CD can also be divided into a localized andmulticentric type. About 90% of the localized type belongsto the HV subgroup, as seen in our patient, and almost allof the multicentric type is histologically the PC subtype.CD can develop anywhere that lymphoid tissue isfound, most commonly in the mediastinum (60%), butalso in the abdomen, neck, lung, and retroperitoneum.Less than 4% of cases present as a lymph nodal mass inthe axilla [5]. Patients with the HV type are usuallyasymptomatic, as our patient was, whereas patients withthe PC type typically present with a broad variety ofsymptoms such as fever, weight loss, generalized lymph-adenopathy, night sweats, and hepatosplenomegaly. Asalso demonstrated in our case, localized CD is normallycured after excision of the tumor with an excellent prog-nosis and 5-year survival of approximately 100%. On theother hand, successful treatment of the multicentrictype often requires multimodal management includingradiotherapy, chemotherapy, and surgery. The progno-sis is generally less favorable [4].In most cases of CD, as in our patient, US shows ahypoechoic, well-circumscribed homogeneous mass lesion[6]. In all reported cases, the longitudinal to transverse axisratio of involved lymph nodes was more than 2 and wassignificantly higher in benign than in malignant lymphnodes [7]. Color Doppler findings are characteristic for thediagnosis of CD: prominent peripheral vascular prolifera-tion in the node is not seen in healthy or reactive lymphnodes and is absent from lymph nodes affected by malig-nancy. Reactive lymph nodes are more likely to preserve anormal vascularity pattern with central hilar vessels,whereas lymph nodes in patients with CD show bizarrenew blood vessels in the periphery due to neovas-cularization, as in our patient [8]. Histologically, poly-morphous lymphoreticular infiltrates containing numerouscapillaries are seen at the periphery of the lymph node. Ma-lignant lymph nodes typically present a mixed vascular dis-tribution including both central and peripheral flow.These US and Doppler findings, although nonspecific,seem to be characteristic for the diagnosis of this uncom-mon disease entity and may help to differentiate this be-nign process from reactive lymph nodes and nodalmetastases. These US findings must be proven to be effi-cacious, and larger studies of patients with CD are re-quired to determine the role of US and sonoelastographyin this group. Whether the distribution of nodal vascular-ity and Doppler flow characteristics can help to achieve abetter understanding of CD must be assessed.As in our case, CD is difficult to diagnose based on as-pirate material. FNAC as the initial investigation methodmay be misleading because no specific cytomorphologicalcriteria for a definitive diagnosis have been described, norare there any cytomorphological features pathognomonicfor the disorder [9].Another technique for preoperative axillary node diag-nosis is US-guided core biopsy. Although more expensiveFigure 3 CT scan shows enlarged lymph node (arrow) in theleft axilla.Figure 4 Macroscopic aspect of affected lymph nodemeasuring 4.5 cm.Wagner and Maden BMC Research Notes 2013, 6:97 Page 3 of 4http://www.biomedcentral.com/1756-0500/6/97and invasive, resulting in a higher complication rate, corebiopsy has the advantage of sampling the nodal tissuemore extensively than using FNAC. Using core needle bi-opsy and excision biopsy, all cases reported in the litera-ture were diagnosed as CD [9]. As in our patient, coreneedle biopsy was superior to FNAC and gave the correctdefinitive diagnosis.The differential diagnoses of an axillary mass includemetastases, lymphoid neoplasms such as Hodgkin’slymphoma and non-Hodgkin lypmphoma, and a numberof reactive, inflammatory, and nonmalignant conditionssuch as rheumatoid arthritis, Wiskott-Aldrich syndrome,tuberculosis, sarcoidosis, syphilis, and other disorders ofimmune regulation in patients with acquired immune de-ficiency syndrome and Kaposi’s sarcoma. Because of itsvariable clinical presentation, CD should be considered asa differential diagnosis of any enlarged lymph node.ConclusionIn conclusion, although it is probably not possible to ren-der a definitive diagnosis of CD based on US findings, thepresence of a hypoechoic lymph node with many promin-ent peripheral vessels on Doppler sonogram should atleast raise the diagnostic possibility. This case report high-lights the difficulty in diagnosing unicentric CD in FNAsamples. Core needle biopsy, which usually achieves thefinal diagnosis, should be given preference. As shown inour case report, unicentric CD should be a differentialdiagnosis of an enlarged lymph node, especially in asymp-tomatic and young patients. Surgical removal of the af-fected lymph node is curative in localized HV-type CD.Confirmation of CD should be based upon the combin-ation of clinical, sonographic, CT, and histopathologicalfindings.ConsentWritten informed consent was obtained from the patientfor publication of this manuscript and accompanyingimages. A copy of the written consent is available for re-view by the Editor-in-Chief of this journal.In this original case report, we first describe the find-ings and imaging features of Castleman’s disease basedon gray-scale ultrasonography (US), color Doppler US,sonoelastography US, and contrast-enhanced dynamiccomputed tomography. The description of the sono-graphic findings in this unique case of Castleman’sd i s e a s eof the axilla will certainly advance our understanding ofthis illness.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsNW and ZM performed the clinical work, data collection, and data analysis.Both authors read and approved the final manuscript.Author details1Department of Obstetrics and Gynecology, Marienhospital Essen,Hospitalstrasse 24, Essen 45329, Germany.2Department of Obstetrics andGynecology, University Hospital Frankfurt, Theodor Stern Kai 7, Frankfurt60590, Germany.Received: 10 September 2012 Accepted: 8 March 2013Published: 15 March 2013References1. Castleman B: Records of the Massachusetts General Hospital-weeklyclinicopathological exercises (case 40011). N Engl J Med 1954, 250:26–30.2. Castleman B, Iverson L, Menendez VP: Localized mediastinal lymphnodehyperplasia resembling thymoma. Cancer 1956, 9(4):822–830.3. Dham A, Peterson BA: Castleman disease. Curr Opin Hematol 2007,14(4):354–359.4. van Rhee F, Stone K, Szmania S, Barlogie B, Singh Z: Castleman disease inthe 21st century: an update on diagnosis, assessment, and therapy.Clin Adv Hematol Oncol 2010, 8(7):486–498.5. Yildirim H, Cihangiroglu M, Ozdemir H, Kabaalioglu A, Yekeler H, Kalender O:Castleman's disease with isolated extensive cervical involvement.Australas Radiol 2005, 49(2):132–135.6. Khashab MA, Canto MI, Singh VK, Ali SZ, Fishman EK, Edil BH, Giday S: Arare case of peripancreatic Castleman's disease diagnosedpreoperatively by endoscopic ultrasound-guided fine needle aspiration.Endoscopy 2011, 43(Suppl 2):E128–E130.7. Baruah BP, Goyal A, Young P, Douglas-Jones AG, Mansel RE: Axillary nodestaging by ultrasonography and fine-needle aspiration cytology inpatients with breast cancer. Br J Surg 2010, 97(5):680–683.8. Raniga S, Shah C, Shrivastava A, Amin P, Patel P: Doppler findings incastleman disease- a rare case. Indian J Radiol Imaging 2006, 16:127–130.9. Ghosh A, Pradhan SV, Talwar OP: Castleman's disease - hyaline vasculartype - clinical, cytological and histological features with review ofliterature. Indian J Pathol Microbiol 2010, 53(2):244–247.doi:10.1186/1756-0500-6-97Cite this article as: Wagner and Maden: An unusual unifocalpresentation of Castleman’s disease in a young woman with a detaileddescription of sonographic findings to reduce diagnostic uncertainty: acase report. BMC Research Notes 2013 6:97.Submit your next manuscript to BioMed Centraland take full advantage of: • Convenient online submission• Thorough peer review• No space constraints or color ﬁgure charges• Immediate publication on acceptance• Inclusion in PubMed, CAS, Scopus and Google Scholar• Research which is freely available for redistributionSubmit your manuscript at www.biomedcentral.com/submitWagner and Maden BMC Research Notes 2013, 6:97 Page 4 of 4http://www.biomedcentral.com/1756-0500/6/97

Axillary node staging by ultrasonography and fine-needle aspiration cytology in patients with breast cancer.

Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy. Clin Adv Hematol Oncol

Doppler findings in castleman disease- a rare case. Indian J Radiol Imaging

Giday S: A rare case of peripancreatic Castleman's disease diagnosed preoperatively by endoscopic ultrasound-guided fine needle aspiration. Endoscopy 2011, 43(Suppl 2):E128–E130.

Kalender O: Castleman's disease with isolated extensive cervical involvement. Australas Radiol

Peterson BA: Castleman disease. Curr Opin Hematol

Records of the Massachusetts General Hospital-weekly clinicopathological exercises (case 40011).

VP: Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer

Springer - Publisher Connector

Wagner and Maden BMC Research Notes 2013, 6:97
http://www.biomedcentral.com/1756-0500/6/97CASE REPORT Open AccessAn unusual unifocal presentation of Castleman’s
disease in a young woman with a detailed
description of sonographic findings to reduce
diagnostic uncertainty: a case report
Norbert Wagner1* and Zerrin Maden2Abstract
Background: Castleman’s disease is a rare lymphoproliferative disorder. It typically presents as mediastinal masses
and causes a wide range of clinical symptoms. Histologically, Castleman’s disease is classified as either a hyalinic
vascular or plasma cell variant. The prognosis mainly depends on the histological type and broadly varies. We
herein report our sonographic findings in a patient with Castleman’s disease, including gray-scale ultrasonography,
color Doppler ultrasonography, and sonoelastography ultrasonography, which have not been previously reported in
the literature. These findings allowed for a preoperative diagnosis and avoidance of overly aggressive therapy.
Case presentation: A 28-year-old European female patient with unicentric Castleman’s disease of hyalinic vascular
type (HV) restricted to the axilla was referred to us because of a 4-month history of a painless, solitary mass located
in the left axilla. The patient’s medical history was unremarkable.
Conclusion: Castleman’s disease is a pathologic entity of unknown etiology and pathogenesis. In this case report
of unicentric HV-type CD, we demonstrate that typical sonographic findings can lead to a preoperative diagnosis of
Castleman’s disease. Core needle biopsy usually allows for a final diagnosis and helps to avoid unnecessary
operations and overtreatment.
Keywords: Castleman’s disease, Giant lymph node hyperplasia, Ultrasonography, Core needle biopsyBackground
Castleman’s disease (CD), or giant lymph node hyperpla-
sia, is a rare lymphoproliferative disorder that typically
presents as mediastinal masses. It was first described by
Castleman and colleagues as a localized mass of medias-
tinal lymphoid follicles in 1954. Two years later, it was
defined as a pathologic entity of unknown etiology and
pathogenesis [1,2].
Clinically, CD may be localized with no major symp-
toms and present as a solitary mass or swelling, or it
may be a generalized, symptomatic disease with fever,
weight loss, anemia, hepatosplenomegaly, and general-
ized lymphadenopathy.* Correspondence: norbertwausu@yahoo.de
1Department of Obstetrics and Gynecology, Marienhospital Essen,
Hospitalstrasse 24, Essen 45329, Germany
Full list of author information is available at the end of the article
© 2013 Wagner and Maden; licensee BioMed
Creative Commons Attribution License (http:/
distribution, and reproduction in any mediumHistologically, the disease is also classified into two
separate subtypes: the hyalinic vascular (HV) variant
(80%–90% of cases) and the plasma cell (PC) variant
(10%–20% of cases). Intermediate and mixed types have
also been reported.
The prognosis mainly depends on the histological type
and shows a broad variety. Treatment can range from
curative surgery for the solitary form to the use of ste-
roids, monoclonal antibodies, chemotherapy, and radio-
therapy for the multicentric type [3].
We herein report on a 28-year-old female patient with
unicentric CD restricted to the axilla. We describe the
findings and imaging features of gray-scale ultrasonog-
raphy (US), color Doppler US, sonoelastography US, and
contrast-enhanced dynamic computed tomography (CT).
A pathway to a preoperative diagnosis, management of
the disease, and the clinical course are presented. A reviewCentral Ltd. This is an Open Access article distributed under the terms of the
/creativecommons.org/licenses/by/2.0), which permits unrestricted use,
, provided the original work is properly cited.
Figure 1 Color Doppler sonogram shows peripheral vascular
flow within an ovoid hypoechoic axillary lymph node.
Figure 2 Histopathologic specimen shows a continuum of
abnormal germinal centers (GC) with prominent CD 23-positive
follicular dendritic cells (dense black brown staining) and
subtle vascular proliferation (original magnification, ×200). MZ,
mantle zone.
Wagner and Maden BMC Research Notes 2013, 6:97 Page 2 of 4
http://www.biomedcentral.com/1756-0500/6/97of the literature and differential diagnoses are also
presented.
Case presentation
A 28-year-old European female patient was referred to
us because of a 4-month history of a painless, solitary
mass located in the left axilla. She had no accompanying
complaints, history of fatigue, night sweats, or weight
loss. Her medical history was unremarkable.
On routine physical examination, a solitary enlarged
lymph node was detected in the left axilla in the absence of
any breast pathology. No generalized lymphadenopathy or
other organomegaly was noted. Peripheral blood counts
and the erythrocyte sedimentation rate were within
normal limits. Interestingly, the levels of lymphoprolifera-
tive markers such as serum soluble IL-2R, beta 2-
microglobulin, and immunoglobulins were also normal;
however, the C-reactive protein level was slightly increased.
The lymph node in the left axilla measured 4 cm and
was mobile, nontender, and soft in consistency. US
examination of the breast and axilla was performed with
an iU22 (Philips Healthcare, Bothell, WA) and ProSound
7 (Aloka, Hitachi, Zug, Switzerland) using a 12-MHz lin-
ear array transducer.
High-frequency, high-resolution gray-scale US revealed
a well-defined, uniformly hypoechoic, ovoid axillary mass,
38 × 17 × 28 mm in size. The longitudinal diameter was
greater than the transverse diameter with a longitudinal to
transverse axis ratio of more than 2. A hyperechoic fatty
hilum could not be detected and was totally replaced by
cortical thickening. Although soft in consistency, the le-
sion could only be slightly deformed by compression with
the transducer. Color Doppler flow was performed with
optimized color Doppler parameters set at a low wall filter
(80–100 Hz) and low velocity scale (pulse repetition fre-
quency, 1000 Hz). Color gain was adjusted dynamically to
maximize depiction of blood vessels while avoiding
artifactual color noise. Bizarre and multifocal peripheral
flow was detected, whereas central or central perihilar flow
was not revealed (Figure 1). A three-dimensional and
multislice imaging scan with the capability of reproducing
high-resolution images confirmed these B-mode findings,
but could not provide additional important information.
Spectral Doppler analysis along the periphery of the node
showed both arterial and venous pulse wave patterns. The
blood flow profile of the arteries indicated a broad range in
the resistance index, pulsatility index, and peak systolic vel-
ocities varying from low to high pulsatility. Thus, no fur-
ther information could be drawn on these indices.
Sonoelastography US confirmed the clinical examin-
ation findings: the lesion was characterized by soft tissue
with some less elastic regions of higher stiffness.
An US-guided fine needle biopsy with multiple pas-
sages of the needle tip through the nodal cortex wasmade to sample as much of the nodal cortex as possible.
Fine needle aspiration cytology (FNAC) only revealed a
mixed population of small and large lymphoid cells. In
particular, prominent vascularity with hyalinized capillaries
was not detected. The FNAC results were subsequently
reported as “negative for malignant cells,” and histo-
pathologic examination of the lymph node was advised.
Therefore, US-guided core needle biopsy using a 14-G
automated gun was performed, and a diagnosis of HV-
type CD was confirmed: microscopic examination revealed
many variably sized hyperplastic follicles, progressive vas-
cular proliferation, and hyalinization (Figure 2).
Figure 4 Macroscopic aspect of affected lymph node
measuring 4.5 cm.
Wagner and Maden BMC Research Notes 2013, 6:97 Page 3 of 4
http://www.biomedcentral.com/1756-0500/6/97A multislice CT scan of the head, thorax, and abdomen
was subsequently performed and allowed for the exclusion
of multicentric type CD. The lymph node in the left axilla
on CT was described as a well-circumscribed, homoge-
neous mass lesion with moderate to intense enhancement
and rapid washout (Figure 3). The patient underwent open
biopsy by a surgical gynecologist, and the enlarged axillary
lymph node was completely excised (Figure 4). The post-
operative course was uneventful, clinical follow-ups
were unremarkable, and there has been no evidence of
recurrence.
Discussion
CD is a rare, benign lymphoproliferative disorder of un-
known etiology. The two main hypotheses for its develop-
ment are an abnormal immune response and viral
infection. Human herpes virus 8 and interleukin 6 are
regarded to be linked to the pathogenesis [4]. Microscopic-
ally, as stated above, two histological subtypes are known:
the HV type and PC type. Depending on the clinical pres-
entation, CD can also be divided into a localized and
multicentric type. About 90% of the localized type belongs
to the HV subgroup, as seen in our patient, and almost all
of the multicentric type is histologically the PC subtype.
CD can develop anywhere that lymphoid tissue is
found, most commonly in the mediastinum (60%), but
also in the abdomen, neck, lung, and retroperitoneum.
Less than 4% of cases present as a lymph nodal mass in
the axilla [5]. Patients with the HV type are usually
asymptomatic, as our patient was, whereas patients with
the PC type typically present with a broad variety of
symptoms such as fever, weight loss, generalized lymph-
adenopathy, night sweats, and hepatosplenomegaly. As
also demonstrated in our case, localized CD is normally
cured after excision of the tumor with an excellent prog-
nosis and 5-year survival of approximately 100%. On the
other hand, successful treatment of the multicentric
type often requires multimodal management includingFigure 3 CT scan shows enlarged lymph node (arrow) in the
left axilla.radiotherapy, chemotherapy, and surgery. The progno-
sis is generally less favorable [4].
In most cases of CD, as in our patient, US shows a
hypoechoic, well-circumscribed homogeneous mass lesion
[6]. In all reported cases, the longitudinal to transverse axis
ratio of involved lymph nodes was more than 2 and was
significantly higher in benign than in malignant lymph
nodes [7]. Color Doppler findings are characteristic for the
diagnosis of CD: prominent peripheral vascular prolifera-
tion in the node is not seen in healthy or reactive lymph
nodes and is absent from lymph nodes affected by malig-
nancy. Reactive lymph nodes are more likely to preserve a
normal vascularity pattern with central hilar vessels,
whereas lymph nodes in patients with CD show bizarre
new blood vessels in the periphery due to neovas-
cularization, as in our patient [8]. Histologically, poly-
morphous lymphoreticular infiltrates containing numerous
capillaries are seen at the periphery of the lymph node. Ma-
lignant lymph nodes typically present a mixed vascular dis-
tribution including both central and peripheral flow.
These US and Doppler findings, although nonspecific,
seem to be characteristic for the diagnosis of this uncom-
mon disease entity and may help to differentiate this be-
nign process from reactive lymph nodes and nodal
metastases. These US findings must be proven to be effi-
cacious, and larger studies of patients with CD are re-
quired to determine the role of US and sonoelastography
in this group. Whether the distribution of nodal vascular-
ity and Doppler flow characteristics can help to achieve a
better understanding of CD must be assessed.
As in our case, CD is difficult to diagnose based on as-
pirate material. FNAC as the initial investigation method
may be misleading because no specific cytomorphological
criteria for a definitive diagnosis have been described, nor
are there any cytomorphological features pathognomonic
for the disorder [9].
Another technique for preoperative axillary node diag-
nosis is US-guided core biopsy. Although more expensive
Wagner and Maden BMC Research Notes 2013, 6:97 Page 4 of 4
http://www.biomedcentral.com/1756-0500/6/97and invasive, resulting in a higher complication rate, core
biopsy has the advantage of sampling the nodal tissue
more extensively than using FNAC. Using core needle bi-
opsy and excision biopsy, all cases reported in the litera-
ture were diagnosed as CD [9]. As in our patient, core
needle biopsy was superior to FNAC and gave the correct
definitive diagnosis.
The differential diagnoses of an axillary mass include
metastases, lymphoid neoplasms such as Hodgkin’s
lymphoma and non-Hodgkin lypmphoma, and a number
of reactive, inflammatory, and nonmalignant conditions
such as rheumatoid arthritis, Wiskott-Aldrich syndrome,
tuberculosis, sarcoidosis, syphilis, and other disorders of
immune regulation in patients with acquired immune de-
ficiency syndrome and Kaposi’s sarcoma. Because of its
variable clinical presentation, CD should be considered as
a differential diagnosis of any enlarged lymph node.
Conclusion
In conclusion, although it is probably not possible to ren-
der a definitive diagnosis of CD based on US findings, the
presence of a hypoechoic lymph node with many promin-
ent peripheral vessels on Doppler sonogram should at
least raise the diagnostic possibility. This case report high-
lights the difficulty in diagnosing unicentric CD in FNA
samples. Core needle biopsy, which usually achieves the
final diagnosis, should be given preference. As shown in
our case report, unicentric CD should be a differential
diagnosis of an enlarged lymph node, especially in asymp-
tomatic and young patients. Surgical removal of the af-
fected lymph node is curative in localized HV-type CD.
Confirmation of CD should be based upon the combin-
ation of clinical, sonographic, CT, and histopathological
findings.
Consent
Written informed consent was obtained from the patient
for publication of this manuscript and accompanying
images. A copy of the written consent is available for re-
view by the Editor-in-Chief of this journal.
In this original case report, we first describe the find-
ings and imaging features of Castleman’s disease based
on gray-scale ultrasonography (US), color Doppler US,
sonoelastography US, and contrast-enhanced dynamic
computed tomography. The description of the sono-
graphic findings in this unique case of Castleman’s disease
of the axilla will certainly advance our understanding of
this illness.
Competing interests
The authors declare that they have no competing interests.
Authors’ contributions
NW and ZM performed the clinical work, data collection, and data analysis.
Both authors read and approved the final manuscript.Author details
1Department of Obstetrics and Gynecology, Marienhospital Essen,
Hospitalstrasse 24, Essen 45329, Germany. 2Department of Obstetrics and
Gynecology, University Hospital Frankfurt, Theodor Stern Kai 7, Frankfurt
60590, Germany.
Received: 10 September 2012 Accepted: 8 March 2013
Published: 15 March 2013
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doi:10.1186/1756-0500-6-97
Cite this article as: Wagner and Maden: An unusual unifocal
presentation of Castleman’s disease in a young woman with a detailed
description of sonographic findings to reduce diagnostic uncertainty: a
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An unusual unifocal presentation of Castleman’s disease in a young woman with a detailed description of sonographic findings to reduce diagnostic uncertainty: a case report

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