Classic central nervous system Hodgkin lymphoma masquerading as left sphenoid wing meningioma- A case report

Abstract

Introduction and importance

Classic Hodgkin Lymphoma with an incidence of 2–3 cases per 100,000 population affects the Central Nervous System in 0.02 % of cases (Gerstner et al., 2008; Brice et al., 2021; Morawa et al., 2007). CNS lymphoma, contributing to 0.22 % of Central Nervous System tumors, is the uncommon extra-nodal manifestation of Hodgkin's Disease (Brice et al., 2021; Henkenberens et al., 2014). It affects the nervous system secondary to systemic lymphoma or the relapse of the disease (Gerstner et al., 2008). Only 17 cases of CNS lymphoma are reported which were limited to the CNS at the time of diagnosis (Paul et al., 2017). Only two cases of Dural-Based Hodgkin Lymphoma were reported in the literature (Paul et al., 2017).

Case presentation

We are reporting the third case of dural-attached extra-axial secondary CNS Hodgkin Lymphoma in a 33 years old female, which appeared and was operated as Left Sphenoid Wing Meningioma.

Clinical discussion

Only 17 cases of CNS lymphoma are reported which were limited to the CNS at the time of diagnosis (Paul et al., 2017). Only two cases of Dural-Based Hodgkin Lymphoma were reported in the literature (Paul et al., 2017). Our case consists the third case of dural attached Classic CNS Hodgkin lymphoma and the first case of Classic CNS Hodgkin Lymphoma locating in the Sphenoid Wing.

Conclusion

It is important to differentiate CNS Hodgkin Lymphoma from other types of brain tumors especially when it resides in an unusual location because the treatment of CNS Hodgkin Lymphoma is mainly combined chemo-radiotherapy than surgical intervention.

Highlights

• •CNS lymphoma is an uncommon extranodal manifestation of Hodgkin's disease.
• •Classic Hodgkin's lymphoma affects the central nervous system as a result of relapse or systemic disease.
• •Only 17 cases of primary central nervous system lymphoma are reported in the literature.
• •Our case is the first classic Hodgkin lymphoma located in the sphenoid ridge.

1. Introduction

Hodgkin lymphoma with two discrete features of Classic Hodgkin Lymphoma and Nodular Lymphocyte Predominant Hodgkin Lymphoma has an incidence of 9000 cases per year [6]. It's rare for Hodgkin disease to affect the central nervous system (0.02 %) [1,3]. CNS involvement is secondary to systemic disease and only 17 cases of primary CNS Hodgkin lymphoma are reported in recent decades [5].

Based on Ann Arbor's classification of lymphomas, Hodgkin lymphoma is classified into four stages. Extra-nodal involvement happens in stage IV which commonly affects the lung, bone marrow, liver, and soft tissue and CNS involvement is rare [2].

Here we report a case of CNS Hodgkin Lymphoma with an unusual location in the Left Sphenoid Wing. The case was diagnosed as Left Sphenoid Meningioma based on imaging findings, but the histopathology findings were consistent with classic Hodgkin lymphoma. This case has been reported in line with SCARE criteria [8].

2. Case presentation

A 33-year-old Asian female presented in the emergency department with complaints of persistent generalized headache for four days. Headache was associated with vomiting and blurring of vision in the left eye. On further inquiring, she mentioned about mild fever at night for which she was taking paracetamol. But there was no history of night sweats and pruritus.

She had no previous medical history of any chronic disease. Her family history was insignificant. Clinical examination was also unremarkable. The patient was alert and oriented with no neurological signs.

Computed tomography (CT) was performed and showed evidence of approximately 3.8 × 3.6 × 1.8 cm lesion in the middle cranial fossa (likely extra-axial) along the greater sphenoid wing with disproportionate edema in the right temporoparietal lobe causing significant mass effect (Fig. 1A,B).

Fig. 1.

A: CT head bone window hypertrophy of left sphenoid wing

B: CT head without contrast. And an irregularly marginated and heterogeneously enhancing mass lesion was seen in the middle cranial foci along the greater sphenoid wing

C: MRI head with contrast axial view. Heterogeneously enhancing extra-axial mass lesion along the anterior aspect of the left middle cranial fossa.

D: MRI head with contrast coronal view. Heterogeneously enhancing lesion based on greater wing of left sphenoid bone.

E: MRI head T2WI coronal view showing extensive edema surrounding the lesion.

Magnetic resonance imaging (MRI) showed Left sphenoid wing meningioma (Fig. 1C, D, E).

A computed tomography (CT) scan of the neck, chest, abdomen, and pelvis showed no evidence of other tumor masses.

The patient underwent left pterional craniotomy for resection of the lesion. The excised specimen consisted of a fragment of tan-white membranous soft tissue. The post-op course was uneventful. Histopathology came and mentioned Classical Hodgkin lymphoma. Immunohistochemistry confirmed CD15 and CD30 positivity in the Reed-Sternberg cells (Fig. 3A, B, C, D).

Fig. 3.

A: H&E stained high magnification of the lymphocytic nodule showing Hodgkin cells (small arrow), Reed-Stenberg cells (large arrow), and mummified cells (arrowhead).

B: Immunohistochemically stain of CD30 which highlights Hodgkin cells and Reed-Stenberg cells with Golgi-dot classical positivity.

C: Immunohistochemical stain of CD15 which also highlights Hodgkin cells and Reed-Sternberg cells

D: Immunohistochemical staining of MUM-1 with classic nuclear staining of Hodgkin and Reed-Sternberg cells.

The whole-body PET scan which showed Hypermetabolic focus in the left breast very suspicious for primary breast carcinoma, two to three tiny nonspecific mild hypermetabolic focus in the right breast, multiple hypermetabolic bony lesions are suspicious for metastasis/lymphomatous involvement, small nodes in the left neck, left supraclavicular region and retroperitoneum showing uptake (Fig. 2).

Fig. 2.

Whole body PET scan showing multiple hyper metabolic bony lesions.

Ultrasound-guided biopsy of breast confirmed benign breast tissue with no evidence of malignancy.

Bone marrow biopsy and a lumbar puncture, which were performed with suspicion of systemic lymphoma with involvement of the neurocranium were suspected, both showed neither lymphoma nor other malignant cells.

The case was discussed in lymphoma MDT which suggested chemotherapy [ICE (Rituximab, Ifosmafide, Carboplatin, Etoposide) and DHAP (Rituximab, Dexamethasone, Cytarabine, Cisplatine] with an interim PET scan.

Repeat PET scan was done after 2 cycles of chemotherapy and revealed complete metabolic resolution of retroperitoneal lymph nodes, breast, lesions, and a marked reduction in the metabolic activity of bone lesions.

The patient completed four cycles of chemotherapy and she is doing well without any neurological deficits. She is discharged home with a follow-up appointment with hematology. It's worth mentioning that post operative MRI showed complete resection of the lesion (Fig. 4A, B) In each step patient received information about her condition.

Fig. 4.

A: Post-op MRI with contrast axial view: complete resection

B: Post-op MRI with contrast coronal view: complete resection.

3. Discussion

CNS Hodgkin Lymphoma is a rare disease defined either as a lesion limited to the CNS at the time of diagnosis or as a consequence of systemic disease [5,2]. The neurological manifestations of this disease are secondary to metastasis, paraneoplastic phenomenon, or treatment-related which happens in 15 % of Hodgkin Disease. Risk factors for CNS involvement are relapsing disease (most common), family history of HL, immune compromise, infection with EBV, and male gender [1,7,4]. There are three mechanisms for secondary CNS involvement of HL through direct extension, meningeal and hematogenous spread [7].

.According to the Ann Arbor classification, CNS lymphoma is divided into four stages. In stage one, the lesion is limited to a single lymph node and in stage four (15 %–30 %) it involves extra nodal tissues (Table 1) [2]. CNS involvement in Hodgkin Lymphoma is rare and happens in 0.02 % of cases [[1], [2], [3]].

Table 1.

Ann arbor classification of CNS lymphoma [2]:

Stages	Stages description
Stage I	A single lymph-node is involved
Stage II	More than one lymph-nodes are involved (confide to one side of the diaphragm)
Stage III	Lymph nodes on both sides of the diaphragm are involved, including eventually the spleen
Stage IV	One or more areas of extranodal involvement
A	Absence of B symptoms
B	Presence of stage B symptoms (night sweats, fever lasting at least a week, and weight loss of more than 10 % of body weight)
C	One extranodal involvement contiguous to a lymph node

In the majority of cases, the lesions appear as periventricular lesions and extra-axial location is the rarest manifestation of the disease. 17 cases of primary CNS Hodgkin lymphoma are reported in recent decades [5]. Among the cases, two presented as extra-axial and attached to the dura lesions. The first one was reported in 1980 as falx-cerebral dural-based lymphoma and the second one was reported in 2016 as a left parietal lesion attached to the dura [5].

Our case presents an unusual location of CNS Hodgkin Lymphoma which presented as Left Sphenoid Wing Meningioma. The lesion is in a unique location that considering the radiologic features we operated the case as meningioma of the middle cranial fossa. The Histopathology report is consistent with Classic Hodgkin's Lymphoma. Furthermore, a thorough investigation with a whole-body PET scan showed multiple hypermetabolic bone lesions consistent with stage IV Hodgkin's disease. Bone Marrow Biopsy and lumbar puncture were negative.

The patient was treated with chemotherapy [ICE (Rituximab, Ifosmafide, Carboplatin, Etoposide) and DHAP (Rituximab, Dexamethasone, Cytarabine, Cisplatine] with an interim PET scan. After completing two cycles of chemotherapy PET scan was negative for extracranial HL.

4. Conclusion

Central Nervous System involvement in Hodgkin Lymphoma is a rare presentation and most of them are a result of metastasis or relapse. Central Nervous System Lymphoma is a rare manifestation of this disease.

Our case discussed the unusual location of CNS Hodgkin Lymphoma as a result it is important to consider it in the differential diagnosis from other brain tumors. As compared to other types of brain tumors the management of CNS Hodgkin Lymphoma is mainly combined chemo-radiotherapy than surgical intervention.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

The manuscript has got ethical review exemption from the Ethical Review Committee (ERC) of our institution, as case reports are exempted from review according to the institutional ethical review committee's policy.

Funding

No financial support was provided for this study.

Author contribution

Khalida Walizada: writing, data collection, editing.

Dr. Bara Abdulhay Ishaq Wazwaz writing case presentation and histopathology report.

Dr. Ghanem senior consultant neurosurgery operated and managed the case.

Guarantor

The corresponding author is the guarantor of article.

Research registration number

Not applicable.

Conflict of interest statement

None.