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Year : 2013  |  Volume : 9  |  Issue : 2  |  Page : 287-289

Pregnancy and cavernous sinus syndrome in diffuse large B-cell lymphoma

1 Department of Gynaecology and Obstetrics, the First Affiliated Hospital of Soochow University, Suzhou, China
2 Department of Haematology, the First Affiliated Hospital of Soochow University, Suzhou, China
3 Department of Neurology, the First Affiliated Hospital of Soochow University; Institute of Neuroscience, Soochow University, Suzhou, Jiangsu, China

Date of Web Publication13-Jun-2013

Correspondence Address:
Jian-Ming Hu
Department of Gynaecology and Obstetrics, Xiao-Wei Hu, Department of Neurology, The First Affiliated Hospital of Soochow University, No.188 Shizi Street, Suzhou
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.113388

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 > Abstract 

Diffuse large B-cell lymphoma (DLBCL) usually present with rapidly growing lymph nodes or extra-nodal masses. Central nerve system involvement and pregnancy are rare in DLBCL. Here, we report an unusual case of DLBCL with cavernous sinus syndrome during pregnancy. A 24-year-old woman presented cavernous sinus syndrome as the initial presentation during pregnancy. Magnetic resonance imaging (MRI) revealed enlargement of bilateral cavernous sinus. Tonsil and ovary biopsy indicated malignant lymphoma-DLBCL. Bone marrow smear showed infiltration by tumor cells. The case delivered a viable baby by cesarean section and then took chemotherapy of rituxan, cyclophosphamide, adriamycin, eldisine and dexamethasone. The disease deteriorated rapidly, especially after the pregnancy was terminated. Fortunately, a complete response was achieved after six cycles of chemotherapy. With the accumulation of clinical practice of such cases, we would be able to recognize minimal symptoms of DLBCL at the beginning and confirm the most suitable timing of the initiation of chemotherapy during pregnancy.

Keywords: Cavernous sinus syndrome, diffuse large B-cell lymphoma, pregnancy

How to cite this article:
Wang J, Chen XC, Hu JM, Hu XW. Pregnancy and cavernous sinus syndrome in diffuse large B-cell lymphoma. J Can Res Ther 2013;9:287-9

How to cite this URL:
Wang J, Chen XC, Hu JM, Hu XW. Pregnancy and cavernous sinus syndrome in diffuse large B-cell lymphoma. J Can Res Ther [serial online] 2013 [cited 2020 Feb 26];9:287-9. Available from: http://www.cancerjournal.net/text.asp?2013/9/2/287/113388

 > Introduction Top

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of malignant lymphoma. Large B-cell lymphomas mostly occur in the elderly, predominally in males. We report here an unusual case of DLBCL with cavernous sinus syndrome as the initial presentation in a young female during pregnancy.

 > Case Report Top

A 24-year-old woman, at week 30 of pregnancy, was admitted with the chief complaint of sudden onset diplopia. The positive signs were a left fixed dilated pupil, ptosis and complete external ophthalmoplegia; right lateral rectus palsy; decreased sensation of bilateral forehead. Cranial magnetic resonance imaging (MRI) revealed enlargement of bilateral cavernous sinus three days before the admission [Figure 1]. Gadolinium-based contrast agents were avoided because of pregnancy. This case was diagnosed with cavernous sinus syndrome. Inflammation, thrombosis of venous sinus or malignant cancer infiltration were considered as the cause.
Figure 1: Bilateral enlargement of cavernous sinus on T2 MRI

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During hospitalization, full blood counts showed hemoglobin 77 g/L and platelet 61 × 109/L. Lactate Dehydrogenase (LDH) was 1039 U/L. The enlarged spleen (13.4 cm at the longest axis) and stable fetal status without malformation were demonstrated on ultrasonography. Left 3 degree antiadoncus was found. Histology pathological examination of a specimen from tonsil biopsy indicated malignant lymphoma-DLBCL [Figure 2]. The immunophenotype was characteristic (CD20 +, CD79a +, Bob1+, Ki-67 + and CD3-, CD43-, Bcl-6-, CD10-, MUM1-, CK-, CK5/6-, TDT-, EBV-). Primary DLBCL of tonsil was therefore diagnosed.
Figure 2: Diffuse large B-cell lymphoma in left tonsil (H and E stain, 400 × magnification)

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At later gestational stages chemotherapy is often feasible and should not be delayed in this situation. However, the patient disagreed on terminating pregnancy and taking chemotherapy. Dexamethasone was applied for improvement of fetal lung maturity. At 32 weeks of gestation the patient underwent cesarean section. A viable baby weighing 1,700 g was delivered. The baby was initially treated in the neonate intensive-care unit for 2 weeks and developed well later. After delivery, the patient's conditions got worse rapidly and she was supported by assisted ventilation. Antiadoncus, anemia and thrombocytopenia aggravated progressively. She was noted to have newly bilateral palpable breast masses, neck and groin masses. Positron emission tomographic computed tomography (PET-CT) with fluorine-18-labelled fluorodeoxyglucose (18 F-FDG) revealed glycometabolism uptake in many areas of the body, including brain, both ovaries and breasts [Figure 3]. Right ovary pathological examination supported the diagnosis of DLBCL. Bone marrow smear [Figure 4] and biopsy samples revealed infiltration by tumor cells. These findings were considered as indication of disseminated malignant lymphoma. Stage IVB highly-grade malignant non-Hodgkin's lymphoma (NHL) was then diagnosed (High risk on the international prognostic index).
Figure 3: Glycometabolism uptake (bright colour) in many areas of the body On PET-CT

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Figure 4: Diffuse large B-cell lymphoma on bone marrow smear before chemotherapy (Wright– Giemsa stain, 1,000 × magnification)

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Chemotherapy was started with Rituxan, cyclophosphamide, adriamycin, eldisine and dexamethasone (R-CHOP). Red cell and platelet were transfused as supportive therapy. A complete response confirmed by bone marrow smear investigation was achieved after the second cycle of treatment [Figure 5]. After six cycles of R-CHOP therapy, the patient obtained a good remission of clinical symptoms, except that adduction movement of the left eye was slightly affected. It is suggested that the patient should take bone marrow transplantation.
Figure 5: Remission of diffuse large B-cell lymphoma on bone marrow smear after 2-cycle R-CHOP therapy (Wright– Giemsa stain, 1,000 × magnification)

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 > Discussion Top

Cavernous sinus syndrome is defined as the involvement of at least two of the third, fourth, fifth, or sixth cranial nerves, or involvement of only one of them, combined with a radiologically confirmed cavernous sinus lesion. [1] Cavernous sinus involvement is rare in NHL, and as the initial presentation of NHL is extremely rare. [2],[3],[4] Our case presented bilateral cavernous sinus syndrome in combination with cavernous sinus lesions on MRI. Based on PET-CT and good response to chemotherapy, we demonstrated that malignant lymphoma disseminated to cavernous sinus. In a few cases of malignant lymphoma with cavernous sinus involvement, most cavernous sinus lesions were infiltrated by lymphoma from the adjacent skull base and paranasal sinuses. [2],[4] Our case was similar to another two patients with cavernous sinus syndrome caused by NHL dissemination to the brain. [3],[5] The high-grade malignant NHL in this case is rapid progressive and considerably invasive, but can occur with minimal symptoms at the beginning, presenting initially as cavernous sinus syndrome. Furthermore, even isolated oculomotor nerve palsy could be the initial manifestaion of DLBCL. [4] In view of the above, we may conclude that although NHL with cavernous sinus involvement is a rare condition, it should be considered in the differential diagnosis in patients, who develop oculomotor abnormalities and imaging signs of cavernous sinus lesions.

Lymphoma in pregnancy, although rare, poses a substantial risk to both mother and fetus. [6] NHL with pregnancy often have unusual manifestations and the diagnosis is frequently delayed. NHL with pregnancy in our case presented cavernous sinus involvement as the initial symptom but developed progressively, in accordance with previous study. [7] Pregnant women with high-grade aggressive lymphoma are more likely to have a fulminant course always with a poor outcome because of delayed diagnosis or insufficient therapy. [6] And in these patients, even a short delay in diagnosis could result in clinically significant disease progression. [6] It was no doubt that a few weeks delay in diagnosis and therapy resulted in the rapid deterioration of the disease in our case. Additionally, the course of NHL seemed remarkably accelerate post partum in this case. Hormonal changes during pregnancy might stabilize the lymphoma cell proliferation until delivery. Previous studies demonstrated that during pregnancy hormonal changes, greater progesterone exposures, lead to a reduction in B-cell lymphopoiesis and inhibit the development of B-cell NHL. [8] So it must be emphasized that therapeutic delay should be avoided in pregnancy-related NHL. However, in practice, patients are often in dilemma. Merimsky et al. once reported a case of NHL with pregnancy and monolateral cavernous sinus involvement. The baby was healthy after cesarean section. After delivery, the patient started the chemotherapy and radiotherapy, unfortunately died of the complication with leukopenia. [9] Another pregnant woman with high-grade B-cell NHL gave a birth to one healthy baby before she began chemotherapy with CHOP, as our case did. That patient had complete remission at 2-year follow-up. [10] During pregnancy, rituximab with CHOP for aggressive NHL should be combined with intensified regimens for highly aggressive subtypes. [6] Given with the bad conditions, our case did not take intensified medications except for R-CHOP. Fortunately, a complete response to R-CHOP has been obtained even in short term. Bone marrow transplantation should be the best for the patient after six cycles of R-CHOP.

To our knowledge, this is the first report of pregnancy-associated high-grade malignant DLBCL with involvement of bilateral cavernous sinus and successful pregnant outcome. With the accumulation of clinical practice of such cases, we would be able to recognize minimal symptoms of malignant NHL at the beginning and confirm the most suitable timing of the initiation of chemotherapy.

 > Acknowledgement Top

We thank Dr. Su-Ya Kang for valuable help with pathological examinations.

 > References Top

1.Lee JH, Lee HK, Park JK, Choi CG, Suh DC. Cavernous sinus syndrome: Clinical features and differential diagnosis with MR imaging. AJR Am J Roentgenol 2003;181:583-90.  Back to cited text no. 1
2.Williams Z, Norbash A, Goode RL. Cavernous sinus syndrome caused by a primary paranasal sinus non-Hodgkin's lymphoma. J Laryngol Otol 1998;112:777-8.  Back to cited text no. 2
3.Abalo-Lojo JM, Gonzalez F, Pereiro-Zabala I. Metastatic B-cell lymphoma of the cecum masquerading as Tolosa-Hunt syndrome. Can J Ophthalmol 2007;42:323-5.  Back to cited text no. 3
4.Sato H, Hashimoto T, Yoneda S, Hirabayashi K, Oguchi K, Higuchi K. Lymphoma as a cause of isolated oculomotor nerve palsy. J Clin Neurosci 2011;18:1256-8.  Back to cited text no. 4
5.Rasper M, Kesari S. Burkitt lymphoma presenting as a rapidly evolving cavernous sinus syndrome. Arch Neurol 2008;65:1668.  Back to cited text no. 5
6.Brenner B, Avivi I, Lishner M. Haematological cancers in pregnancy. Lancet 2012;379:580-7.  Back to cited text no. 6
7.Gelb AB, van de Rijn M, Warnke RA, Kamel OW. Pregnancy-associated lymphomas. A clinicopathologic study. Cancer 1996;78:304-10.  Back to cited text no. 7
8.Prescott J, Lu Y, Chang ET, Sullivan-Halley J, Henderson KD, Clarke CA, et al. Reproductive factors and non-Hodgkin lymphoma risk in the California Teachers Study. PLoS One 2009;4:e8135.  Back to cited text no. 8
9.Merimsky O, Reider I, Rahmani R, Chaitchik S. Pregnancy and cavernous sinus involvement in a patient with primary non-Hodgkin's lymphoma of bone. Isr J Med Sci 1990;26:520-4.  Back to cited text no. 9
10.Silva PT, de Almeida HM, Principe F, Pereira-Leite L. Non-Hodgkin lymphoma during pregnancy. Eur J Obstet Gynecol Reprod Biol 1998;77:249-51.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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