Abstract
A 57-year-old man was referred to our department with a mass in the sphenoid sinus. Surgical removal of the tumor was performed. However, a testicular mass was found that showed rapid growth. He had undergone inguinal orchiectomy. Five days after the urological surgery, he noticed visual disturbances and bilateral visual loss. Ophthalmological examination revealed total blindness, and magnetic resonance imaging revealed sphenoid mass growth. The patient underwent emergent removal of the tumor, and a diagnosis of malignant lymphoma was made. A final diagnosis of mantle cell lymphoma (MCL) in the testis and sphenoid sinus was made. After receiving treatment with intravenous corticosteroids and chemotherapy for lymphoma, his left vision completely recovered. Although his right vision was lost, he returned to normal social activities. This is the first report in the literature on MCL developing in the sphenoid sinus presenting with bilateral blindness and ipsilateral recovery.
Keywords: blindness, mantle cell lymphoma, sphenoid sinus, testicular tumour
Introduction
Nasal and paranasal sinus lesions may cause visual disturbances and often require immediate surgical interventions. Various diseases such as cysts, infections, and tumors can cause visual disturbances. Malignant lymphomas also develop in the nose and paranasal sinuses. Although rare, it can cause visual disturbances [1, 2]. Mantle cell lymphoma (MCL) is rare. This report describes a case of systemic MCL arising in the sphenoid sinus that caused rapid visual loss.
Case report
A 57-year-old man was referred to our department with a mass in the sphenoid sinus. He first noticed headache, photophobia, and body discomfort on the left side, and then visited a neurological clinic. The patient did not complain of any other symptoms, including visual loss. Cervical lymphadenopathy and any mass in Waldeyer’s ring were not observed. Blood test results, including soluble interleukin-2 receptor levels, were normal. Magnetic resonance imaging (MRI) revealed a mass in the sphenoid sinus; therefore, the patient was referred to our department 6 days after developing the initial symptoms. Nasal endoscopy revealed swelling of the anterior wall of the sphenoid sinus, with purulent nasal discharge on the right side (Fig. 1). The patient did not complain of any visual symptoms. Plain computed tomography (CT) revealed a soft-density mass in the sphenoid sinus that expanded to the nasopharynx, sphenoid sinus, and orbit. Surgical removal and pathological examination were performed. However, the patient suddenly noticed an enlarged left scrotal sac. Two days later, the patient visited a urologist and underwent contrast-enhanced CT. Abdominal imaging showed tumors in the left and left adrenal glands. Therefore, testicular cancer with adrenal metastasis was suspected. The next day, the patient underwent left high testicular resection by urologists. He was discharged 5 days after urological surgery; however, the next day, he noticed sudden blindness in both eyes. The patient visited an ophthalmologist and visual loss was noted. Magnetic resonance imaging (MRI) of the orbit revealed that the sphenoid sinus mass grew, expanding to both orbits and attached to both optic canals (Fig. 2). Based on this imaging study, the sphenoid mass caused a sudden visual disturbance, and the patient was referred to our department again. On the second visit, the sensation of light was completely lost in both eyes. Light reflexes were absent and papillary edema was observed in both eyes. Testicular cancer metastasis was initially suspected; however, a pathological diagnosis was not made during this period. Emergency surgery was performed under general anesthesia to relieve visual loss and estimate the mass. The right sphenoid wall is swollen and pulsatile. The anterior wall was incised and the sphenoid sinus was filled with a grayish tumor (Fig. 3). Malignant lymphoma was diagnosed based on a rapid pathological diagnosis. To relieve the optic nerve damage, the mass was removed as far as possible. The left sphenoid sinus was then opened. The tumor expanded from the right side, and purulent discharge filled the cavity, but there was no tumor expansion to the lateral wall of the left sphenoid sinus.
Figure 1.
Endoscopic image of the right nasal cavity. The tumour protruded into the sphenoethmoidal recess (S: nasal septum, M: middle turbinate, I: inferior turbinate, T: tumour).
Figure 2.
Gadolinium-enhanced MRI before surgery. The tumour protruded into the optic canal bilaterally, and this tumour also pressed and/or invaded the optic nerve on the right side (arrow).
Figure 3.
Surgical image of the right nasal cavity. The sphenoid wall was filled with grayish tumours. The diagnosis of malignant lymphoma was made.
Histological examination of the tumor revealed diffuse infiltration of moderately large abnormal lymphocytes. Immunostaining revealed CD5 and cyclin D1 expression. The percentage of Ki-67-positive cells was found to be 80%. In contrast, CD, CD10, EBER-ISH, CD56, CD30, AE1/AE3, and CAM5.2 were negative (Fig. 4). The blastoid variant was diagnosed based on conspicuous fission images. Testicular tumors were found to have the same pathology. Based on these findings, systemic MCL was diagnosed.
Figure 4.
Histology of this tumour. Diffuse infiltration of moderately large abnormal lymphocytes was observed (HE: hematoxylin–eosin staining). Immunostaining showed CD5 and cyclin D1 positivity. The rate of Ki-67-positive cells was 80%. The blastoid variant was diagnosed based on the conspicuous fission image.
The next morning, he noticed the left visual recovery and recognized finger movements. Five hundred milligrams of hydrocortisone succinate sodium were administered for 3 days, and the steroid dose was reduced and continued for 9 days. He underwent chemotherapy with six courses of R-CHOP and two courses of HD-MTX at the hematology department, and complete remission of the disease was achieved. The right vision had never recovered, whereas the left visual acuity had improved to 0.9 a month after the beginning of chemotherapy. One year after these episodes, the patient was disease-free and returned to normal social activities.
Discussion
Malignant lymphoma can cause various neural symptoms anywhere in the affected body parts [1–4]. Any lesion, including malignant lymphoma in the sphenoid sinus, may cause acute visual disturbances [1, 2]. Lymphomas may develop in the paranasal sinus; the maxillary sinus is the most common site, followed by the ethmoid sinuses [5–8]. Diffuse large B-cell lymphoma is the most common histological type, followed by follicular lymphoma and natural killer/T-cell lymphoma [5–8]. Lymphomas that develop in the sphenoid sinuses are rare, and diffuse large B-cell lymphoma is a common histological finding [1, 2, 6]. MCL developing in the paranasal sinus is far rarer, and its development in the sphenoid sinus has never been reported [9, 10]. This is the first case in terms of the site of origin and histological findings.
Unfortunately, the right blindness had never recovered, whereas the left blindness had recovered. During surgery, the tumor invaded the lateral wall of the right sphenoid sinus, suggesting direct invasion of the optic nerve. In contrast, no tumor expansion to the lateral wall of the left sphenoid sinus was observed. Concerning visual disturbances caused by sphenoid lesions, sphenoidal cysts frequently cause visual disturbances. However, cyst opening leads to immediate visual recovery. In this case, no direct invasion of the left optic nerve was considered, so drainage of the sphenoid sinus and successful administration of corticosteroids and chemotherapy for lymphoma were performed, resulting in vision recovery on the left side.
Finally, the present case suggests that sphenoid sinus lesions can always cause visual impairment because the sphenoid sinus is close to the optic nerve. The same situation may occur in the posterior ethmoid sinus; therefore, when encountering a sinus lesion, clinicians should always consider ophthalmological complications. Once visual impairment occurs, imaging and prompt surgical intervention are essential.
Acknowledgements
The authors thank Dr. Kanako Ogura, Dr. Hiroyuki Terukina, and Dr. Mizuki Iino for providing accurate pathological examinations.
Contributor Information
Yosuke Kanaya, Department of Otolaryngology-Head and Neck Surgery, Juntendo Nerima Hospital, Nerima-ku Takanodai 3-1-10, Tokyo 177-0033, Japan.
Atsunobu Tsunoda, Department of Otolaryngology-Head and Neck Surgery, Juntendo Nerima Hospital, Nerima-ku Takanodai 3-1-10, Tokyo 177-0033, Japan.
Kanako Saigo, Department of Otolaryngology-Head and Neck Surgery, Juntendo Nerima Hospital, Nerima-ku Takanodai 3-1-10, Tokyo 177-0033, Japan.
Kumiko Tanaka, Department of Otolaryngology-Head and Neck Surgery, Juntendo Nerima Hospital, Nerima-ku Takanodai 3-1-10, Tokyo 177-0033, Japan.
Akihisa Yoshikawa, Department of Otolaryngology-Head and Neck Surgery, Juntendo Nerima Hospital, Nerima-ku Takanodai 3-1-10, Tokyo 177-0033, Japan.
Satoko Kubo, Department of Otolaryngology-Head and Neck Surgery, Juntendo Nerima Hospital, Nerima-ku Takanodai 3-1-10, Tokyo 177-0033, Japan.
Fumihiko Matsumoto, Department of Otorhinolaryngology, Juntendo University Graduate School of Medicine, Tokyo, Japan.
Conflict of interest
All authors have no conflict of interest related to this paper.
Funding
All authors received no funding.
Ethical approval
This study was approved by the ethical institutional review board of Juntendo University Nerima Hospital approved this report (S 22-12).
Consent
Written informed consent was obtained from the patient.
Guarantor
Yosuke Kanaya and Atsunobu Tsunoda.
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