Clinical Characteristics Of Childhood Lymphoma Onset With Cranial Nerve Involvement And Literature Review

ObjectiveChildhood lymphoma onset with cranial nerve involvement is insidiously occurring,misdiagnosis rate is high,and related reports are few.In this study,clinical data of these children were retrospectively analyzed and literature review was conducted to summarize the clinical characteristics of pediatric lymphoma with such special onset,so as to improve the understanding of pediatric lymphoma with rare onset,avoid misdiagnosis,achieve early diagnosis and treatment,and improve the prognosis of children.MethodA retrospective analysis conducted on the clinical data of childhood lymphoma hospitalized in the Qilu Hospital of Shandong University from January 2005 to June 2021,5 patients onset with cranial nerve involvement who finally diagnosed with lymphoma were collected on the basis of the different onset clinical manifestations.At the same time,through retrospectively analyzed the literature retrieval which has the same onset manifestation of pediatric lymphoma at home and abroad,in order to summarize the clinical data,auxiliary examination results,treatment and prognosis of pediatric lymphoma in this rare disease form and then synthetically analyzed these dates.ResultFive children onset with cranial nerve involvement were admitted to Qilu Hospital of Shandong University,all of these children were diagnosed as non-Hodgkin’s lymphoma ultimately,including 4 males and 1 female,with onset ages ranging from 4 to 16 years.Affected nerve is oculomotor nerve,optic nerve,auditory nerve,facial nerve and olfactory nerve respectively.Case 1 started with drooping eyelids and blurred vision.Case 2 presented with progressive vision loss and binocular blindness.The onset of case 3 was progressive hearing loss.Case 4 was caused by facial palsy and blurred vision.Case 5 was initiated by anosmia.The time from the clinical symptom occurred to diagnosis ranged from 20 to 40 days.Bone marrow cytology in 3 children suggested lymphomatous infiltration.There were no tumor cells in all 4 out of 5 children’s cerebrospinal fluid,but only one child’s cerebrospinal fluid protein and lactic acid were elevated.Skull,cavernous sinus and dura were involved in 1 case,skull,sinus,cavernous sinus and optic nerve were involved in 1 case,and nasopharyngeal masses were found in 2 cases.Among the 5 children,4 received chemotherapy,and their symptoms were relieved after treatment;Case 5 received radiotherapy,chemotherapy and surgery,but finally died during chemotherapy.A total of 28 cases of childhood lymphoma with cranial nerve involvement were collected through literature search,including 23 males and 5 females,aged 3 to 17 years.Among the 28 patients,there was 1 Hodgkin lymphoma and 27 non-Hodgkin lymphoma.Among the 28 children,19 were admitted for abnormal vision,and the frequency of cranial nerve involvement was as follows:optic nerve in 14 cases,oculomotor nerve in 12 cases,abducens nerve in 4 cases,trochlear nerve in 3 cases.The clinical manifestations were:decreased vision,blurred vision,ptosis,diplopia,blindness and so on.Facial nerve palsy was present in 8 cases.Trigeminal neuralgia was present in 1 case.The time from onset to diagnosis ranged from 1 day to 1 year.19 out of the 28 children had done the marrow puncture and in 7 of them,the result was indicated that lymphoma was infiltrating bone marrow.Cerebrospinal fluid results were described in 12 children,and abnormal cerebrospinal fluid results were found in 4 of them.Cranial imaging results were found in 25 of the 28 children,and the frequency of involvement of abnormal craniocerebral sites was as follows:skull in 10 cases,sphenoid sinus in 10 cases,cavernous sinus in 7cases,meningeal in 4 cases,brain parenchyma in 4 cases,cranial nerve in 4 cases,maxillary sinus in 4 cases,ethmoid sinus in 4 cases.Brain imaging showed nasal mass in 2 cases and parsella sella mass in 1 case.Among the 28 children,14 received chemotherapy,among which 11 had remission after chemotherapy,and 3 died during chemotherapy.Four children were treated with chemotherapy,radiotherapy and surgery,among which 3 had significant improvement after treatment,and 1 had no improvement in cranial nerve involvement symptoms after 4-month follow-up.4 patients received chemotherapy and radiotherapy,1 patient received chemotherapy and surgery,1 patient received radiotherapy,all of the clinical symptoms were all relieved.Two children were treated with surgery alone,and their prognosis was uncertain.1 child died due to refusal of treatment after diagnosis;The treatment and prognosis of 1 patient are unknown.ConclusionDue to the low incidence and hidden onset of childhood lymphoma onset with cranial nerve involvement,the early stage of the disease is difficult to distinguish from other diseases,and the misdiagnosis rate is high.The possibility of malignant lymphoma should be considered for cranial nerve damage with acute onset and progressive aggravation in clinic.Imaging examination,bone marrow cytology and cerebrospinal fluid examination are helpful for diagnosis.The gold standard of diagnosis is pathological tissue biopsy.If early identification and active comprehensive treatment can be carried out,the prognosis is generally good.