Swati Singh & Mohammad Javed Ali
Purpose: To perform a literature review on the primary malignant epithelial tumors (PMET) involving the lacrimal drainage system (LDS).
Methods: The authors performed a PubMed search of all articles published in English on primary malignant epithelial tumors of the lacrimal sac and the nasolacrimal duct. Data analyzed include prevalence, demographics, clinical presentation, histopathological subtypes, management modalities, outcomes, and recurrence rates.
Results: Malignant epithelial tumors of the lacrimal sac are commonly diagnosed in the fifth to seventh decades of life (mean age: 58 years) and have no gender predilection. Typical clinical presentation includes epiphora (84%) and lacrimal fossa mass lesion (78%). Pain (9%) and blood-stained epiphora (4%) were inconsistent findings. Among the PMET, squamous cell carcinomas (SCCs) were the most common (61%) followed by transitional cell carcinomas (TCC; 15%), and mucoepidermoid carcinoma (MEC; 7%). Complete surgical excision followed by radiotherapy (59%) was the preferred modality of management, and only 18% required orbital exenteration. Of the 331 tumors with available treatment details, 85% survived at a mean follow–up of 33 months. The overall recurrence rate following treatment was 20% (65/331). MECs of the lacrimal sac had the best while adenoid cystic carcinoma had the worst prognosis.
Conclusion: Blood-stained epiphora was not the classical presentation for primary epithelial malignancies of the LDS. No validated staging system exists for lacrimal drainage tumors. There is a need for consistent and detailed reporting in the literature, more so on the histological subtypes and their management.
Ping Wang, Nan Ma, Shaobo Zhang, Xiaona Ning, Chenjun Guo, Qiong Zhang, Qilin Cheng & Yangjun Li
Primary malignant tumours of the lacrimal sac are rare, surgery and radiotherapy may induce substantial side effects for patients. Here, this article reports an innovative technique of interstitial brachytherapy developed for the treatment of malignant lacrimal sac tumours.
Patients and methods
Four patients (male 3, female 1), with an average age of 52.7 years (range 41–72 years), were individually diagnosed with squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma and lymphoma. All patients received Iodine-125 interstitial brachytherapy after surgical resection for malignant lacrimal sac tumours. Visual function examination (vision, intraocular tension, fundus photography, fluorescein angiography, and optical coherence tomography) and CT/MRI/PET-MRI were performed to look for signs of recurrent tumours or metastasis.
Four patients were followed for an average of 28 months (range, 23–37 months). All patients were free from local disease. Their visual function was normal, and CT/MRI did not reveal any tumour recurrence.
Iodine-125 interstitial brachytherapy can be used as an alternative to wide excision or exenteration of these tumours. There was good local control, reasonable maintenance of vision, and good cosmesis.
Ingvild Ramberg, Peter Bjerre Toft, Steffen Heegaard
Malignant tumors of epithelial origin are the most frequent neoplasms of the lacrimal drainage system (LDS). A total of 539 cases are reported in the literature from 1960 to 2019. LDS carcinoma is a disease of the middle-aged and elderly population, with a median age at diagnosis of 57 years. Overall, there is a slight male predominance. Reported risk factors are chronic inflammation, LDS papilloma, and infection with oncogenic viruses. Symptoms of an LDS carcinoma resemble those of benign, inflammatory diseases, with epiphora from obstruction of the LDS as the most frequently encountered symptom. The median time from symptoms to diagnosis is 12 months and even longer for patients reporting epiphora as the only initial symptom. This diagnostic delay leads to a substantial fraction of patients with locally advanced tumors and lymph node spread at the time of diagnosis. Surgery with adjuvant radiotherapy is the treatment of choice for most patients; however, the extension and aggressiveness of the disease and the patient’s general health and preferences determine the definite treatment. Five-year overall survival is reported to range from 61 to 87.6%. A large tumor size and positive lymph node status correlate with a decreased overall and disease-free survival.
David S. Curragh, Craig James & Dinesh Selva
Purpose: To report a case of a lacrimal sac tumour identified at the time of endoscopic dacryocystorhinostomy and describe a swinging inferior turbinate approach to the nasolacrimal duct (NLD) to facilitate an en-bloc excision of the lacrimal drainage apparatus.
Methods: An 88-year-old male presented with a 6-month history of epiphora and mucocele. Endonasal DCR was performed for nasolacrimal duct obstruction (NLDO). Intraoperatively, a biopsy was performed of abnormal appearing lacrimal sac mucosa, following opening of the lacrimal sac. Subsequent definitive management was performed via a combined external and endoscopic approach using a swinging inferior turbinate approach to the NLD to facilitate an en-bloc excision of the lacrimal drainage apparatus, without removal of the bony medial wall of the maxillary sinus.
Results: The excised lacrimal drainage showed insitu and invasive squamous cell carcinoma of the canaliculi and lacrimal sac with focal divergent neuroendocrine and sebaceous differentiation. There was no local tumour recurrence or metastatic spread at 3 months of follow-up.
Conclusions: We describe a swinging inferior turbinate approach to the NLD to facilitate an en-bloc excision of the lacrimal drainage apparatus, without entering the maxillary sinus. We believe this modified technique is a useful option to consider in the management of tumours of the lacrimal drainage apparatus.
David S. Curragh, Alkis J. Psaltis, Neil C. Tan & Dinesh Selva
Purpose: To report a case of a lacrimal sac tumour and describe a prelacrimal approach to the maxillary sinus to excise the nasolacrimal duct in its management. To our knowledge, this approach has not been adapted to remove the nasolacrimal duct for the management of pathological processes involving the nasolacrimal system.
Methods: A 58-year-old female patient presented with a 6-month history of epiphora. A lacrimal sac mass was identified, and a biopsy revealed squamous cell carcinoma. Surgical excision was performed via a combined external and endoscopic prelacrimal approach.
Results: A prelacrimal approach to the maxillary sinus to excise the nasolacrimal duct in combination with an external approach facilitated an en-bloc excision of the nasolacrimal apparatus. This allowed preservation on the entire inferior turbinate following reconstitution of the lateral nasal wall at the completion of the procedure.
Conclusion: The endoscopic endonasal prelacrimal approach to the maxillary sinus is a useful method to approach and excise the nasolacrimal duct in the management of nasolacrimal pathology.
Zhang, Rui; Qian, Jiang; Meng, Fengxi; Yuan, Yifei; Bai, Mengying; Bi, Yingwen; Xu, Binbin; Yuan, Yiqun; Hong, Fujian
Purpose: To determine the effect of a staged procedure in the treatment of primary lacrimal sac epithelial malignancy.
Methods: This is a retrospective case series of 18 consecutive patients with primary lacrimal sac epithelial malignancy treated at an orbital tumor referral center between 2002 and 2017. Study was conducted in compliance with the Declaration of Helsinki. All patients underwent biopsy of the mass to confirm the diagnosis pathologically. Chemotherapy concurrent with radiotherapy was delivered to the patients to reduce and concrete the tumor prior to surgery. En bloc resection of the lacrimal sac malignancy and nasolacrimal duct was followed.
Results: Eleven patients were male and 7 patients were female. The median follow-up time was 72.2 months. Nine patients had squamous cell carcinoma, 7 poorly differentiated carcinoma, 1 transitional cell carcinoma, and 1 adenoid cystic carcinoma. After chemotherapy and radiotherapy, the tumor volume was reduced significantly (p < 0.0001). En bloc resection of the lacrimal sac malignancy was performed in all patients with concurrent partial ethmoidectomy in 8 patients and medial maxillectomy in 5 patients. One patient (5.6%) suffered from adenoid cystic carcinoma died of metastatic disease. Two patients (11.1%) with local recurrence received reoperation, and 1 patient (5.6%) with pulmonary metastasis received gamma knife radiosurgery. These patients are alive with no evidence of tumor. Other patients are alive without evidence of disease at last follow up. No patient had new onset of lymph node enlargement during and after the treatment.
Conclusions: The staged procedure is a promising method for the treatment of primary lacrimal sac epithelial malignancy with no postoperative lymph node metastasis.
Li-juan Zhu, Yu Zhu, Shang-chen Hao, Pei Huang, Lin-lin Wang, Xiu-hong Li, Li-hong Lian & Ke Zhang
To summarize clinical experience on the diagnosis and treatment for malignancy originating from the dacryocyst.
We retrospectively analyzed clinicopathological data from 12 cases that were diagnosed with malignancy originating from the dacryocyst by histopathological examination in our hospital from 2007 to 2017.
Of the 12 cases with malignancy originating from the dacryocyst, 7 were male and 5 were female, with a mean age of 53 years (range, 4–81). Clinical manifestations included a mass in the dacryocyst area in 12 cases, epiphora in 9 cases, pyoid tears in 2 cases, bloody tears in 3 cases, and redness and swelling in the dacryocyst area in 2 cases. Lymphoma occurred in six cases, malignant melanoma in three cases, embryonal rhabdomyosarcoma in one case, and squamous cell carcinoma in two cases.
Misdiagnosis and missed diagnosis readily occur for malignancy originating from the dacryocyst because its clinical manifestations are diverse. For the suspected patients, it is necessary to perform related examinations. Individualized treatment should be adopted based on pathological types and specific conditions.
Radke, Phillip M.; Herreid, Peter A.; Sires, Bryan S.
Primary extracranial meningioma is a rare tumor, unlike its common intracranial counterpart. Infrequently found in the head and neck region, it can cause local or neurologic symptoms depending on its location and interaction with cranial nerves. While uncommon, it is an important diagnosis to have on the differential of periorbital lesions as surgical removal and prognosis are generally good. The authors present the first report of a patient with primary extracranial meningioma originating in the lacrimal sac fossa.
Xinmao Song, Jie Wang, Shengzi Wang, Weifang Wang, Shuyi Wang, Wenjia Zhu
To investigate the pathology, clinical manifestations, and potential risk factors associated with the prognosis of malignant lacrimal sac tumors. In addition, the treatment outcomes and complications were also evaluated.
Ninety cases of malignant lacrimal sac tumors were retrospectively analyzed at our hospital. Pathological classifications, clinical manifestations, risk factors, and follow-up time were documented. The outcomes and complications were evaluated and compared among the various treatment modalities.
The median follow-up time was 50 months (range, 3–258 months). The 5-year overall survival (OS) and progression-free survival (PFS) for all cases were 85.7 and 77.9%, respectively. The 5-year OS and PFS for 69 cases of squamous cell carcinoma were 87.6 and 76.3%, and which were 80.4 and 72.4% for 21 cases of non-squamous cell carcinoma, respectively. There was no difference of 5-year OS and PFS between squamous cell carcinoma and non-squamous cell carcinoma (p = 0.350 and p = 0.946). Positive lymph node status was associated with worse OS (p < 0.001) and PFS (p = 0.020). For the 23.3% of cases (21/90) treated with the definitive radiotherapy, the outcomes were equivalent to that of surgery combined with radiotherapy, with the incidence of treatment-related visual acuity complication not being significant. The addition of chemotherapy to the treatment course had a marginal and non-significant improvement in OS and distant metastasis-free survival.
Lymph node status was found to be a key factor for prognosis. Advanced tumors could benefit from multimodality treatment, with radiotherapy playing an important role. However, the role of chemotherapy requires further investigation.