Castleman disease in children: Diagnosis and treatment

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Castleman disease in childrenB: DệnihagvnioệnsiTsraunndgtưrơeantgmHeunết

Case Series

CASTLEMAN DISEASE IN CHILDREN:

DIAGNOSIS AND TREATMENT

Dinh Viet Hung¹*, Nguyen Thi Thu Thao¹, Le Tho Duc¹, Nguyen Minh Tuan¹, Dao Trung Hieu¹

DOI: 10.38103/jcmhch.2020.64.11

ABSTRACT

Background: We describe the experiences in diagnosis and results of treatment in pediatric Castleman

disease.

Method: Serial case reports.

Result: From 2016 to 2019, we had 7 cases of pediatric Castleman disease: 3 boys and 4 girl. The

median age at diagnosis was 147 months (121-173 months). Clinical manifestations were found in ﬁve

cases. They were all unicentric Castleman disease (6 abdominal mass, one left infraclavicular mass). All

patients were operated with postoperative period uneventful. The median time of postoperative follow up

was 22.7 months (11-53 months) with no signs of relapse.

Conclusions: Pediatric Castleman disease is a rare benign lymph node hyperplasia, it can be localised

or disseminated. Operation is the treatment of choice for localised Castleman disease.

Key words: Castleman disease, angiofollicular lymph node hyperplasia, giant lymph node hyperplasia,

lymph node hamartoma.

about 10% in the abdominal cavity [4]. This is a rare

disease in children, especially in the abdominal cavity.

I. INTRODUCTION

Castleman disease is a rare disorder of lymph node

proliferation, ﬁrst described by Benjamin Castleman

in 1956, characterized by an increase in the size of

focal or diﬀuse lymph nodes. The disease is usually

divided into 2 types, hypervascular and plasmacytic

patterns based on microscopic pathology. The dis-

ease is also divided into localized or diﬀuse based

on general lesions [1-7]. Clinical manifestations of

Castleman disease vary widely from asymptomatic

to nonspeciﬁc features such as fatigue, weight loss,

anemia and high fever [1,2,3,4,5]. Therefore, diagno-

sis and treatment are often diﬃcult.

II. MATERIALS AND METHODS

We conducted a retrospective review of cases of

Castleman disease being treated at Children’s Hos-

pital 1 from January 2016 to December 2019.

III. RESULTS

There were 7 cases including 3 boys and 4 girls,

with the median age at diagnosis of 147 months

(121-173 months). Five patients were hospitalized

This condition is mainly observed in adults with for clinical manifestations of fatigue, loss of appe-

lesions in the chest accounting for about 70% and only

tite, weight loss, pallor, and fever. For case 3, the

1. Department of Surgery, Children’s Hospital 1 - Received: 2/6/2020; Revised: 10/7/2020;

- Accepted: 4/9/2020

- Corresponding author: Dinh Viet Hung

- Email: viethungcaisan@gmail.com; Phone: 0919655982

Journal of Clinical Medicine - No. 64/2020

Hue Central Hospital

patient had high fever (39ºC) without identiﬁed site treated by intravenous broad-spectrum antibiotics.

of infection except very high CRP, leukocyte 17.3 In the following days the patient continued to have

k/ul with neutrophil accounted for 71% and was

intermittent fever with CRP almost unchanged.

Table 1: Clinical characteristics and laboratory proﬁle

Clinical signs

and symp-

toms

Size of

lesion

(cm)

Age

(month)

CRP (mg/

dl)

Location of

lesion

Patient

Sex

Pathology

Retroperi-

toneal tu-

mor next to giofolli-cular

pancreas-tic lymph node

Castleman dis-

ease with an-

Asymptom-

atic

YVN

Male

139

142

5.2

6.5

head

hyper-plasia

Post-perito-

Retroperito- Castleman

neal tumor

next to

pancreas-tic lymph node

head

neal tumor in-

creased in size

(detected at 4

years of age)

disease with

angio-follicular

TNTA

LTHA

Female

hyper-plasia

Fatigue, pal-

lor, weight

loss. High

Castleman

disease

with angio-

Postperito-

neal tumor

170

fever for 2

190

next to pan- follicular

weeks before

surgery. De-

layed puberty

creas body

lymph node

hyper-plasia

Castleman

disease

with angio-

follicular

lymph node

hyper-plasia

Fatigue, an-

orexia, weight

loss, abdomi-

nal pain

Root of jeju-

nal mesen-

tery

TTD

NTQ

NTL

Male

Female

Male

121

173

143

145

6.2

7.8

3.4

4.4

Castleman

disease

with angio-

follicular

lymph node

hyper-plasia

Abdominal

pain, fatigue,

anemia

Root of the

small bowel

mesen-tery

Castleman

disease

with angio-

follicular

lymph node

hyper-plasia

Anorexia,

exhaustio/

thalasse-mia

Left supra-

clavicular

region

151

178

Castleman

disease

with angio-

follicular

lymph node

hyper-plasia

Root of the

small bowel

mesen-tery

Abdominal

pain

NTUN Female

Journal of Clinical Medicine - No. 64/2020

Castleman disease in childrenB: DệnihagvnioệnsiTsraunndgtưrơeantgmHeunết

One patient had been diagnosed with an intra-

They were all unicentric Castleman disease in-

abdominal tumor at the age of 4 without any treat- cluding 6 cases with abdominal mass, one case with

ment because it was suspected of an adenoma of left supraclavicular mass). The median diameter of

the pancreatic head and showed no clinical mani- the lesion was 5.79 cm (3.4 - 7.8 cm).

festations other than a slow increase in size. One

patient was diagnosed when being treated as diges- ease with pathological results of Castleman disease

tive disorder. with angiofollicular lymph node hyperplasia.

Table 2: Treatment and follow up

All of these cases were localized Castleman dis-

Duration of

follow up

(months)

Preoperative di-

agnosis

Method of

surgery

Early postopera-

tive period

Patient

Outcome

Resection of

Pancreatic tumor tumor/ open

surgery

No evidence

of relapse

YVN

No complication

Resection

of tumor/

laparos-copic

No evidence

of relapse

TNTA

Pancreatic tumor

surgery

Resection of

Tumor next to the

tumor/ open

No more fever, nor-

malization of CRP,

no complication

No evidence

of relapse

LTHA

TTD

head of pancreas

surgery

Mesenteric tumor Resection

suspected of lym- of tumor/

No more abdominal

pain and fatigue,

No evidence

of relapse

phoma

laparos-copic gain of appetite, no

surgery

complication

No more abdominal

pain and fatigue,

gain of appetite, no

more anemia, no

complication

Mesenteric tumor

suspected of Cas-

tleman disease

Resection of

tumor/ open

surgery

No evidence

of relapse

NTQ

NTL

Tumor at the

supraclavicular

region suspected

of Castleman dis-

ease

No more fatigue,

gain of appetite,

normalization of

CRP, no complica-

tion

Resection of

tumor

No evidence

of relapse

No more abdominal

pain, gain of appe-

tite, , normalization

of CRP, no compli-

cation

Mesenteric tumor Resection of

NTUN suspected of Cas- tumor/ open

tleman disease surgery

No evidence

of relapse

At the beginning, Castleman disease was not tics of vascular proliferation found on computed

thought of in the first four patients. Patient 6 had tomography.

a needle biopsy before complete resection but the

The lesions were completely resected in all 7

diagnosis was not confirmed. The remaining cas- cases with both open and laparoscopic surgery de-

es were not performed needle biopsy due to the pending on the location and sizes of the tumors.

location of the tumor as well as the characteris-

All patients recovered well, clinical symptoms

Journal of Clinical Medicine - No. 64/2020

Hue Central Hospital

quickly disappeared right in the ﬁrst few days after symptoms such as enlargement of lymph nodes,

fever, fatigue, weight loss, autoimmune disorders,

recurrent infections, anemia, hypoalbuminemia, in-

creased CRP, etc [4,6,7].

surgery. Antibiotics for cases with fever were dis-

continued as soon as the results of pathological ﬁnd-

ings were obtained and CRP levels decreased to 33

mg/dl on the third postoperative day.

Five out of seven cases in our report showed

symptoms despite the pathological results being

angiofollicular lymph node hyperplasia. Previous

reports have not shown an association of tumor size

with clinical symptoms.

These patients were discharged and monitored

based on clinical manifestations and periodic ul-

trasound examination with a median follow-up

time of 22.7 months (11-53 months). Results at

follow-up showed a physical recovery, gain of ap-

petite as well as the disappearance of symptoms

and no signs of relapse.

In order to diagnose Castleman disease, diagnos-

tic imaging tests such as ultrasound or chest and ab-

domen CT scans are recommended for patients with

symptoms such as fatigue, loss of appetite, anemia,

prolonged fever but not yet found the cause. Screen-

ing ultrasound during routine physical exams is rec-

ommended to detect lesions without clinical mani-

festations.

IV. DISCUSSION

Castleman disease is a benign proliferation of

lymphocytes and cytoplasm, also known by other

names: giant lymph node hyperplasia, lymph

node hamartoma, angiofollicular lymph node

hyperplasia [4].

Until now, surgical removal of the entire lesion is

the ﬁrst-choice method for the treatment of unicentric

Castleman disease. The disease is treated right after

diagnosis, although no studies have shown the ability

and time for the unicentric disease to turn into multi-

centric disease, but the risk of progression to malig-

nancy (lymphoma) has been reported [1,3,5].

The proliferation of blood vessels of the lesion [7]

makes needle biopsy dangerous. On the other hand,

the biopsy results obtained are also very diﬃcult for

deﬁnitive diagnosis. Therefore, surgical removal of

the entire lesion is usually performed without prior

pathological diagnosis.

Since the ﬁrst case with lesions locating in the an-

terior mediastinum in adults was described by Cas-

tleman et al in 1954, an increasing number of cases

have been reported with various locations of the lym-

phatic system. Castleman disease appears to occur in

any location of the body’s lymphatic system, with the

most common site being the anterior mediastinum

accounting for about 70%, only less than 10% in the

abdominal cavity followed by the neck [2,4].

Six out of 7 cases in our report had intra-ab-

dominal lesions, perhaps a coincidence to help us

learn from the diagnosis and treatment of this rare

disease. However, we believe that many cases of

Castleman disease in the mediastinum as well as

many other sites have not been detected due to the

absence of clinical manifestations such as the case

of patient 2 showing that this disease can progress

silently asymptomatic for years.

Although the pathogenesis is still poorly un-

derstood, several reports have noted an association

between clinical manifestations and an increase in

Interleukin 6 [2]. Clinically, the multicentric Castle-

man disease with mainly cytoplasmic pattern in mi-

croscopic examination often presents with systemic

Figure 1: Open surgery to treat Castleman disease

(A,B: Lesions on CT scan and during surgery;

C: Lesion from complete resection of the tumor)

(Patient LTHA).

Journal of Clinical Medicine - No. 64/2020

Castleman disease in childrenB: DệnihagvnioệnsiTsraunndgtưrơeantgmHeunết

All 7 of our cases were indicated for total surgi- found an appropriate answer to the clinical manifes-

cal resection based on computed tomography evalu- tations of the two remaining cases although the path-

ation. During the operation we noted that the sur- ological results of all 4 cases were Castleman disease

gery was relatively uneventful due to the relatively with angiofollicular lymph node hyperplasia.

clear limitation of the lesion, but care must be taken

to reveal and protect important blood vessels, which

in our cases are visceral artery, mesenteric artery

and carotid bundle. Lesions may be in one location

but may include multiple lymph nodes and need to

remove all of them.

It seems that lesions may be removed either by

open or laparoscopic surgery, even though lesions

locating adjacent to important blood vessels can

make laparoscopic surgery more dangerous. Lapa-

roscopic surgery is also helpful for us to locate the

lesion and select the appropriate abdominal route in

operation. Preoperative embolization by intravas-

Figure 3: Microscopic images of Castleman dis-

ease with angiofollicular lymph node hyperplasia

A. Hyperplasia of follicles in the lymph node.

B. “Lollipop” sign (head of arrow)

(Patient LTHA).

Many reports show that for unicentric Castleman

cular intervention - in cases of extensive vascular disease, surgical removal can be completely cured,

hyperplasia and in lesions at unfavorable locations but there are cases of relapses after many years ^(1-7)

– to help a complete resection has been reported [7]. Therefore, it is necessary to monitor long-term for

Figure 2: Laparoscopic surgery in the treatment of cases of surgical removal.

Relapse or unresectable lesions or multicentric

disease can be treated with a variety of therapies

such as corticosteroid monotherapy or chemother-

apy. Other methods currently being applied, such as

interferon alpha, retinoic acid and anti-IL6 mono-

clonal antibodies, also show promising results.

Castleman disease

V. CONCLUSIONS

(Patient TNTA).

Castleman disease is a rare disease in children

with a diverse clinical presentation characterized by

proliferation of lymph nodes with single or diﬀuse

lesions. Complete surgical removal is the treatment

of choice for unicentric Castleman disease.

Ultrasound and computed tomography are eﬀec-

tive means of detecting the disease.

The patients recovered well and there were no

complications. We noted a rapid disappearance of

symptoms in two cases ID 3 and 4 right in the ﬁrst

few days of postoperation, especially the CRP level

of the third case decreased to 33 mg/dl on the third

postoperative day. It seems that the abrupt elimina-

tion of secreting chemicals from the tumors (such as

IL6) is the cause of this recovery but we have not

The outcome of surgery is favorable, but patients

need long-term follow-up to check for a relapse.

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