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Strahlentherapie

und Onkologie Case Study

Complete Remission of a Lymphoma-Associated


Chylothorax by Radiotherapy of the Celiac Trunk
and Thoracic Duct
Johanna Gerstein1, Dorothea Kofahl-Krause2, Jörg Frühauf1, Michael Bremer1

Background: A chylothorax is a rare complication of mostly advanced malignant lymphomas. A case of a refractory chylothorax
unresponsive to chemotherapy and successfully treated with radiotherapy is reported.
Case Report: A 45-year-old woman with recurrent stage IV low-grade follicular non-Hodgkin’s lymphoma and a progressive
chylothorax is described. The CT scans showed bulky lymphadenopathy at the thoracic trunk but no detectable enlargement of
mediastinal lymph nodes. After ineffective pretreatment including chemotherapy and chest drainage, fractionated radiotherapy
to the celiac trunk (20.4 Gy) and the thoracic duct (15 Gy) was performed.
Result: Already after 7.5 Gy a rapid decline of chylothorax was noted and the chest drain could be removed. A complete remission
of the chylothorax could be achieved after 20.4 Gy. During a follow-up of 16 months no recurrence of chylothorax occurred. CT
scans showed nearly complete remission of the lymphadenopathy of the celiac trunk 12 months after radiotherapy.
Conclusion: Radiotherapy with limited total doses is an effective treatment option for lymphoma-associated chylothorax and
should always be taken into consideration, especially in cases unresponsive to chemotherapy.
Key Words: Chylothorax · Non-Hodgkin’s lymphoma · Radiotherapy

Strahlenther Onkol 2008;184:484–7


DOI 10.1007/s00066-008-1840-4

Komplette Remission eines lymphombedingten Chylothorax nach Bestrahlung des Truncus coeliacus und Ductus
thoracicus
Hintergrund: Ein Chylothorax stellt eine seltene Komplikation bei malignen, meist fortgeschrittenen Lymphomen dar. Die Be-
strahlung eines refraktären Chylothorax ohne Ansprechen auf Chemotherapie wird beschrieben.
Fallbericht: Es wird über eine 45-jährige Patientin mit rezidiviertem follikulären Non-Hodgkin-Lymphom Stadium IV und progre-
dientem Chylothorax berichtet. CT-Aufnahmen zeigten eine ausgeprägte Lymphadenopathie im Bereich des Ductus thoracicus,
jedoch ohne Nachweis vergrößerter mediastinaler Lymphknoten. Nach erfolglosen Vorbehandlungen einschließlich Chemothera-
pie und Thoraxdrainage erfolgte eine Bestrahlung des Truncus coeliacus (20,4 Gy) und des Ductus thoracicus (15 Gy).
Ergebnis: Bereits nach 7,5 Gy kam es zu einer raschen Abnahme des Chylothorax, und die Thoraxdrainage konnte entfernt werden.
Eine komplette Remission des Chylothorax wurde nach 20,4 Gy erreicht. Während einer Nachbeobachtungszeit von 16 Monaten
trat kein Rezidiv des Chylothorax auf. 12 Monate nach Bestrahlung war bildgebend eine nahezu komplette Remission der Lymph-
adenopathie im Bereich des Truncus coeliacus nachweisbar.
Schlussfolgerung: Eine niedrigdosierte Bestrahlung ist eine effektive Therapieoption beim lymphomassoziierten Chylothorax und
sollte immer in Erwägung gezogen werden, besonders nach fehlendem Ansprechen auf eine Chemotherapie.
Schlüsselwörter: Chylothorax · Non-Hodgkin-Lymphom · Bestrahlung

1
Department of Radiation Oncology, Hannover Medical School, Germany,
2
Department of Hematology, Hemostasis, Stem Cell Transplantation and Oncology, Hannover Medical School, Germany.

Received: December 4, 2007; accepted: May 28, 2008

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Gerstein J, et al. Successful Radiotherapy of Lymphoma-Associated Chylothorax

Introduction Case Report


A chylothorax is characterized by pleural effusions with A 45-year-old woman initially presented in July 2000 with a
milky white appearance due to a high content of triglycerides stage IVA follicular lymphoma grade 2 with bone marrow in-
(> 110 mg/dl) and presence of chylomicrons. It is a rare com- filtration and enlarged supra- and infradiaphragmatic lymph
plication of Hodgkin’s or non-Hodgkin’s lymphoma (NHL) nodes excluding the mediastinum. Due to a progressive bulky
mostly associated with advanced disease. Three mechanisms disease at the celiac trunk chemotherapy was initiated with
can result in pleural effusions in lymphomas: obstruction of 2-CDA (cladribine, 5 mg/m2 d1–3) combined with mitoxan-
the thoracic duct by mediastinal or retroperitoneal lymph- trone (8 mg/m2 d1 + 2, q4w ×3) in October 2001. The course of
adenopathy, lymphomatous infiltration of the pleura, and disease was complicated due to incompliance. In 2003, after
obstruction of pleural lymphatics by mediastinal lymph- two cycles of highly active therapy with anti-CD20 monoclo-
adenopathy [6]. Due to the high intrinsic radiosensitivity of nal antibody (rituximab) and bendamustine, the patient can-
lymphomas mediastinal radiotherapy represents a valid treat- celed further treatment as well as a planed high-dose therapy
ment option, although clinical data are very limited [12, 13]. with stem cell transplantation in 2004.
Corresponding to the results of the German Hodgkin Study In July 2006, she returned for emergency treatment with
Group [11], additive radiotherapy in the area of bulky disease progressive dyspnea due to an extensive bilateral chylothorax
could be useful for patients with no complete remission after persisting > 2 months (Figure 1a). No signs of ascites were
chemotherapy and is still an integral part within com- found. Fluid analysis of pleural aspiration confirmed the diag-
bined-modality treatment [2, 9]. nosis of chylothorax with presence of chylomicrons. CT scans
The present case report aims at drawing attention to the of the thorax and abdomen revealed bulky lymphadenopathy
potential benefit of radiotherapy for the treatment of persis- at the celiac trunk (12 cm in diameter, Figure 2a) but no de-
tent secondary chylothorax unresponsive to chemotherapy. tectable enlargement of mediastinal lymph nodes or signs of
malignant pleural infiltration. Initial
treatment of the chylothorax consisted
of an oral diet with medium-chain tri-
glycerides (MCTs), two courses of che-
motherapy with bendamustine com-
bined with rituximab due to the
previously good response in 2001. Al-
though the lymph nodes promptly de-
creased in size, no reduction of the mag-
nitude of pleural effusions was noted
necessitating repeated chest punctures
a b
with fluid volumes of 1.5–2.0 l. Mean-
Figures 1a and 1b. CT scans of the thorax showing extensive bilateral pleural effusion before while, the patient had lost 20% of her
onset of radiotherapy (a) with complete remission 4 months after radiotherapy (b). body weight with a body mass index of
Abbildungen 1a und 1b. CT des Thorax mit ausgeprägtem Chylothorax beidseits vor Bestrah- 16. Finally, a chest tube on the left tho-
lungsbeginn (a) und kompletter Rückbildung 4 Monate nach Bestrahlung (b). racic side became necessary with daily
drainage of 1.2–2.5 l of chylous liquid.
Finally, after discussion at the multi-
disciplinary tumor board, decision was
made in favor of radiotherapy. Radio-
therapy was delivered after CT-based
three-dimensional treatment planning
using 23-MV linac photons with anteri-
or-posterior/posterior-anterior (AP/PA)
portals. Radiation fields encompassed
the lymph nodes at the celiac trunk as
a b well as a small strip of the mediastinal
thoracic duct up to the jugular fossa (Fig-
Figures 2a and 2b. CT scans of the abdomen showing bulky lymphadenopathy at the celiac
trunk before onset of radiotherapy (a) and nearly complete remission 12 months after radio- ure 3a). Decision was made against ra-
therapy with 20.4 Gy (b). diation fields limited to the celiac trunk
Abbildungen 2a und 2b. CT des Abdomens mit ausgeprägter Lymphadenopathie auf Höhe des only because up to now, there have been
Truncus coeliacus vor Bestrahlungsbeginn (a) und nahezu kompletter Rückbildung 12 Monate no reports of radiotherapy of chylotho-
nach Bestrahlung mit 20,4 Gy (b). rax in the literature without inclusion of

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Gerstein J, et al. Successful Radiotherapy of Lymphoma-Associated Chylothorax

the mediastinum. Additionally, the possibility of obstruction (two courses R-ICE and BEAM) followed by autologous
of the thoracic duct could not be ruled out despite the lack of PBSCT (peripheral blood stem cell transplantation) in July
detectable mediastinal lymphadenopathy. Initially, single 2007, but the patient died with suspected hepatic progress and
dose was 1.5 Gy due to the field size with five fractions per septic multiorgan failure in December 2007. Of note, no recur-
week. Due to the rapid treatment response (see below) treat- rent pleural effusions occurred during the complete 16 months
ment fields were reduced after 15 Gy to limit exposure of bone of follow-up after radiotherapy.
marrow to irradiation. A boost was given to the macroscopic
tumor at the celiac trunk only to a cumulative total dose of Discussion
20.4 Gy (single dose 1.8 Gy, Figure 3b). A chylothorax is characterized by the accumulation of chyle
Already after 7.5 Gy (five fractions) the magnitude of the in the pleural space and is rarely described in malignant dis-
pleural chyle declined rapidly and the chest drain could be re- ease. Obstruction of the thoracic duct is the most common
moved. After 20.4 Gy pleural effusions had cleared complete- cause of nontraumatic chylothorax. Malignant lymphoma
ly (Figure 1b) and radiotherapy was stopped. Clinically, the constitutes 70% of cases with tumor-associated chylothorax
dyspnea resolved and the body weight increased by 3 kg. [12]. Beyond systemic treatment directed against the caus-
Acute side effects during radiotherapy were not noted. Dur- ative disease several treatment modalities have been estab-
ing a follow-up of 16 months repeated CT scans revealed no lished including oral diet with MCTs, pleurodesis, ligation of
signs of recurrence of the chylothorax as well as a continuous thoracic duct, or even chest tube drainage [4]. Very limited
shrinkage of the infradiaphragmatic bulk as a sign of local con- data are available on the potential role of radiotherapy of a
trol (Figure 2b). refractory chylothorax with only few case reports existing in
7 months later, progressive disease was noted with highly the literature [4, 7, 10]. Earlier reports describe mediastinal
positive FDG-PET (fluorodeoxyglucose positron emission to- radiotherapy as an efficient local treatment of lymphoma-as-
mography) with lesions in the neck, thorax, abdomen and pel- sociated pleural effusions [1, 5, 10, 14]. Assuming obstruction
vis. Therefore, radioimmunotherapy with Zevalin® (ibritu- of the thoracic duct by lymphadenopathy as the causative
momabtiuxetan; 90Y, 800 MBq) was given in December 2006, mechanism of developing a chlyothorax, Daly & Kunkler [7]
but another relapse disease evolved in May 2007. Finally, the proposed mediastinal radiotherapy with 30 Gy in ten frac-
patient agreed to undergo high-dose salvage chemotherapy tions as an appropriate local treatment. They described a pa-
tient with a persistent chylothorax unresponsive to chemo-
therapy. In their case report, CT scans showed extensive
lymphadenopathy in the upper mediastinum. A rapid re-
sponse was noted already after six fractions of radiotherapy
and the chest drain could be removed. During the follow-up
no recurrence of the chylothorax occurred despite a relapse
of the NHL. Bruneau & Rubin [5] reported on mediastinal
radiotherapy in a small series of patients with lymphoma-as-
sociated chylothorax. They treated five patients with doses of
14–19 Gy leading to persistent remission of the chylothorax.
Johnson et al. [10] reported a rapid and persistent resolution
of the chylothorax after mediastinal radiotherapy with a total
dose of 20 Gy (single dose 2.0 Gy). In accordance with these
reports we found a rapid and permanent remission of the chy-
lothorax after irradiation of the celiac trunk (20.4 Gy) and
mediastinal thoracic duct (15 Gy). Radiotherapy was well tol-
erated; however, the intestine is one of the critical organs in
abdominal radiotherapy [3, 8].
a b
In this case report we describe, to our knowledge for the
Figures 3a and 3b. Digitally reconstructed radiographs (DRRs) of the first time, the successful resolution of a chylothorax after ra-
individually shaped treatment fields: initially, large treatment volume diotherapy with lymphadenopathy being present at the celiac
encompassing the celiac trunk and the thoracic duct up to the left trunk only. However, it remains unclear if restriction of target
jugular vein (a), followed by a boost to the bulky lymph nodes at the volume to the celiac trunk had resulted in the same efficacy of
celiac trunk (b).
treatment because we decided to include the mediastinal part
Abbildungen 3a und 3b. Digitale Rekonstruktion (DRR) der individuell of the thoracic duct into the radiation fields.
kollimierten Bestrahlungsfelder: große initiale Bestrahlungsfelder un-
ter Einschluss von Truncus coeliacus sowie Ductus thoracicus bis auf
The fact that it took about 2 months between diagnosis of
Höhe der linken Vena jugularis (a) mit anschließendem Boost auf die refractory pleural effusion and start of effective radiotherapy
Lymphknoten auf Höhe des Truncus coeliacus (b). in this patient highlights the importance of an early multidisci-

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Gerstein J, et al. Successful Radiotherapy of Lymphoma-Associated Chylothorax

plinary approach involving radiation oncologists in the man- 9. Eich HAT, Müller RP. Current role and future developments of radiotherapy
in early-stage favourable Hodgkin’s lymphoma. Strahlenther Onkol 2007;
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10. Johnson D, Klazynski P, Gordon W, et al. Mediastinal lymphangioma and
Conclusion chylothorax: the role of radiotherapy. Ann Thorac Surg 1986;41:325–8.
11. Müller RP, Eich HT. The development of quality assurance programs for ra-
Radiotherapy is a rarely reported but effective treatment of
diotherapy within the German Hodgkin Study Group (HHSG). Introduction,
lymphoma-associated chylothorax. It is well tolerated due to continuing work and results of the radiotherapy reference panel. Strahlen-
the low total doses necessary and can achieve a rapid and per- ther Onkol 2005;181:557–66.
sistent remission of pleural effusions. Radiotherapy should 12. Nair SK, Petko M, Hayward MP. Aetiology and management of chylothorax
in adults. Eur J Cardiothorac Surg 2007;32:362–9.
always be taken into account, especially in cases unresponsive 13. Sakata KI, Satoh M, Someya M, et al. Analysis of local control in patients
to chemotherapy. with non-Hodgkin’s lymphoma according to the WHO classification.
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14. Weick JK, Kiely JM, Harrison EG Jr, et al. Pleural effusion in lymphoma.
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