Lacrimal sac lymphoma: a case series and literature review.

AIM: To study the clinical and pathological characteristics of the lacrimal sac lymphoma, which is rare but it is the major type of non-epithelial malignant tumor in the lacrimal sac region. METHODS: Sixty-four cases of malignant lacrimal sac tumors in our hospital from 1986 to 2020 were retrospectively reviewed. Eight cases of lacrimal sac lymphoma were carefully reviewed. RESULTS: There were five mucosal-associated lymphoid tissue (MALT) lymphomas, one diffused large B-cell lymphoma, one NK/T cell lymphoma, and one mantle cell lymphoma. All eight patients represented symptoms of epiphora with swelling in the lacrimal sac for a certain period of time and showed no signs of systemic involvement at the first time of clinical visits. They had received either chemotherapy or radiotherapy after surgery. Long-term follow-up (from 11 to 220mo) showed that, except one patient with MALT lymphoma died for unknown reasons at 104mo after surgery, the other 7 patients were all alive with no signs of local recurrence, neither in other organs. CONCLUSION: Non-epithelial malignant tumors of the lacrimal sac are rare and lymphoma is the major subtype.

Successful management of adenoid cystic carcinoma of the lacrimal sac with apatinib combined with concurrent chemoradiotherapy: a case report.

Adenoid cystic carcinoma (ACC) of the lacrimal sac is an extremely rare disease with a poor prognosis. There is currently no standard treatment for this malignancy. Radical surgical excision followed by radiotherapy and chemotherapy is the preferred treatment for localized ACC of the lacrimal sac. Apatinib has shown efficacy in recurrent/metastatic ACC. The role of apatinib in combination with concurrent chemoradiotherapy in patients with non-operated localized advanced ACC is not clear. Here, we report a 30-year-old man with a 1-year history of epiphora of the left eye and nasal congestion who was diagnosed as ACC of the lacrimal sac. The computed tomography (CT) scan showed the tumor invading the left orbit and left maxillary sinus. He refused surgical resection and instead received combined apatinib and nedaplatin-based concurrent chemoradiotherapy. He experienced moderate adverse effects such as nausea, hypertension, myelosuppression that were well controlled after symptomatic supportive care. Complete response was observed 3 weeks following the treatment. The patient remained free of disease until the last follow up by December 2020, 22 months after the treatment. This case suggested that combined apatinib and concurrent chemoradiotherapy might be an option for locally advanced ACC patients who are ineligible for or rejective to surgical resection.

Immunoglobulin G4-related lacrimal sac dacryocystocele: A novel case report.

PURPOSE: Immunoglobulin G4-related disease is a systemic fibroinflammatory disease of unknown etiology. Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) can manifest in multiple ways, but lacrimal sac involvement is rare. We present the first case in Chinese population of lacrimal sac IgG4-ROD. OBSERVATIONS: Lacrimal sac IgG4-ROD is rare, and only 9 cases were reported in literature. Despite reported cases in Asian population, mainly in Japan, there was none from the Chinese population or South Asia. Our index case is a 67-year-old Chinese male, who presented with a left insidious nasolacrimal duct swelling mimicking dacryocystocele. Lacrimal sac IgG4-ROD was diagnosed with radiological, serological, pathological and immunohistochemical evidence. The under-reporting of this disease entity may suggest a benign course of such. CONCLUSIONS AND IMPORTANCE: This is the first reported case of biopsy proven lacrimal sac definite IgG4-ROD in Chinese patient on English literature. With the limited cases reported in literature, the pathology of Immunoglobulin G4 immune process in lacrimal sac demands further investigation.

Combined Endoscopic and External Resection of an Inverted Papilloma of the Lacrimal Sac: A Case Report and Review of the Literature.

BACKGROUND: Inverted papillomas (IPs) are benign, locally aggressive neoplasms with a high recurrence rate that most commonly arise from the lateral nasal wall. Rarely, IP can originate from the lacrimal sac and/or nasolacrimal duct (NLD) system. A 58-year-old man presented with chronic epiphora and an enlarging mass inferior to his left medial canthal tendon (MCT) for 2 years. OBJECTIVE: The patients' clinical presentation and surgical management are described. A literature review on IP of the lacrimal sac and NLD system was conducted. METHODS: Case report. RESULTS: We present a case of an IP that arose primarily from the lacrimal sac and grew below the MCT rather than extending above it. This is one of few published case reports detailing the use of a combined endoscopic and external approach for the resection of an IP of the lacrimal sac. CONCLUSION: Combined endoscopic and external resection is a viable approach for complete removal of extensive IP of the lacrimal sac and NLD system. IP arising from the lacrimal sac may grow below the MCT or extend above it as previously reported.

Clinical analysis of 90 cases of malignant lacrimal sac tumor.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Basaloid adenocarcinoma of the lacrimal sac: A rare entity and approach to management.

Presented is a rare case of basaloid adenocarcinoma of the lacrimal sac. Low grade features of this malignancy permitted a conservative surgical approach to maximize functional and cosmetic outcomes. This is retrospective case description of a single 77-year-old-female patient who underwent a dacryocystorhinostomy with silicone tubes at an outside hospital for epiphora, but was referred to the Wills Eye Hospital Oculoplastics Department for a lacrimal sac biopsy showing low-grade carcinoma with basaloid features. She was treated with complete excision of the mass, common canaliculus and distal canaliculi, and the nasolacrimal duct. The associated maxillary, lacrimal, and ethmoidal bones were similarly excised. Frozen sections were sent of the surrounding periosteum, orbital fat, nasal and sinus mucosa, and soft tissues, and all were negative. Reconstruction of the lacrimal system was performed with silicone tube intubation and the defect was closed with large glabellar and mid-facial flaps. One year post-operatively she maintained a normal ocular examination, and her eyelids and silicone tubes were well positioned. Systemic evaluation remained unrevealing. Basaloid adenocarcinoma of the lacrimal sac is relatively rare, and we highlight the role of a more conservative surgical intervention given the high rates of survival and low incidence of progression to metastatic disease when treating this rare malignancy.

Clinical Characteristics of Lacrimal Sac Tumors: Report of Ten Cases

BACKGROUND AND OBJECTIVES: To review our experience with lacrimal sac tumors in an effort to identify features, to evaluate the results of various methods of management, and to compare our data with previous studies. METHODS: We reviewed the medical records of all patients with lacrimal sac tumors who were managed in our institution between January 1990 and December 2015. The pre-operative clinical data, imaging, operation notes, and follow-up records were reviewed for each patient. RESULTS: The study group consisted of four men and six women with a mean age of 47.6 years. Most patients experienced long-standing epiphora, for a mean period of 20 months. Two of the tumors were benign, and eight of them were malignant. The benign tumors were treated with dacryocystectomy. All but one malignant tumor were treated with medial or total maxillectomy. Adjuvant radiotherapy was administered to four patients with malignant tumors. In the eight patients with malignant tumors, the mean follow-up period was 65 months. CONCLUSIONS: Important characteristics of lacrimal sac tumors include dacryocystitis, epiphora, and in some cases, a palpable medial canthal area mass. Wide en bloc resection via medial or total maxillectomy and/or postoperative radiotherapy are proper treatments for malignant lesions of the lacrimal sac.

Transitional cell-type papillary carcinoma of the lacrimal sac manifesting as a medial canthal skin lesion.

A 61-year-old man presented with a 2.5 cm fungating skin lesion above the right medial canthus, accompanied by fluctuance and subtle inferotemporal globe displacement. The tumor had erupted 2 months previously and grown rapidly. Computed tomography (CT) revealed a 4.2 × 2.8 × 2.1 cm exophytic mass of the right orbit, superimposed on chronic dacryocystitis. Incisional biopsy disclosed faulty epithelial maturation sequence with possible basement membrane invasion. The patient underwent wide excision with medial maxillectomy, inferior turbinectomy, ethmoidectomy, and partial rhinectomy. The orbital floor was then reconstructed; a paramedian forehead flap and myocutaneous cheek advancement flap were used to fill cutaneous defects. All surgical margins were negative on both frozen and permanent sections. Definitive histopathology was consistent with transitional cell type papillary carcinoma of the lacrimal sac. Adjuvant radiotherapy was recommended, but the patient has declined further treatment. He remains recurrence-free at one year.

A Case of Juvenile Xanthogranuloma in the Lacrimal Sac

PURPOSE: To report a rare case of juvenile xanthogranuloma in the lacrimal sac. CASE SUMMARY: An 8-year-old female presented to our hospital with epiphora and palpable mass on the left medial canthal area. As a symptom, a sticky and bloody discharge through the punctum had begun on occasion 3 months previously. When pressure was applied on the left medial canthal area, a mucoid discharge through the punctum occurred, and a solid mass was palpable. The mass localized in the lacrimal sac and upper nasolacrimal duct was observed on orbit CT. No invasion into the surrounding tissue was present. The authors of the present study decided to perform endonasal dacryocystorhinostomy, and subsequently a skin-colored mass was found in the lacrimal sac. The histopathological and immunohistochemical diagnosis was histiocytic proliferation, compatible with juvenile xanthogranuloma. Six months later, the patient complained of epiphora recurrence with palpable mass on medial canthal area. The tumor recurrence was confirmed with enhanced orbit CT finding 14 months after the first surgery. A second surgery using endonasal dacryocystorhinostomy was performed to remove the mass.

Primary Rhabdomyosarcoma of the Lacrimal Sac in a 68-year-old Woman

PURPOSE: Rhabdomyosarcoma of the lacrimal sac is extremely rare. There has only been one case in this country, during the 30s, and to our knowledge, there are no reports of primary rhabdomyosarcoma on the lacrimal sac. We report a case of primary rhabdomyosarcoma of the lacrimal sac in a 68-year-old woman METHODS: A 68-year-old female patient presented with tearing of the right eye. A lacrimal sac mass extending to the proximal nasolacrimal duct was detected during the dacryocystorhinostomy, and incisional biopsy was performed. Histopathology revealed a rhabdomyosarcoma of the right lacrimal sac. After systemic chemotherapy, complete excision of the tumor through the medial maxilla was performed. RESULTS: The pathologic diagnosis of the excised mass was embryonal type rhabdomyosarcoma. There was no evidence of tumor recurrence in the lacrimal sac or paranasal sinus during 9 months of follow-up. CONCLUSIONS: Although rhabdomyosarcoma is very uncommon, this case provides the rationale for including it in differential diagnosis of a mass in lacrimal sac.

Management with Radical Excision in Squamous Cell Carcinoma of the Lacrimal Sac

PURPOSE: Squamous cell carcinoma of the lacrimal sac is rare and there is high rate of recurrence after dacryocystectomy. We report successful radical excision of the tumor for the purpose of reducing the recurrence and improving the survival rate. METHODS: A 48-year-old man who had a history of epiphora and medial canthal mass for one year was referred to our clinic. CT scan showed a mass on the lacrimal fossa but there was no evidence of bony destruction or local invasion. We confirmed that the excised mass was squamous cell carcinoma of the lacrimal sac by frozen section. So, radical excision including bony and mucous nasolacrimal duct (NLD), inferior turbinate, and medial maxilla was performed through lateral rhinotomy. RESULTS: Ten months later, there was skin defect and medial canthal deformity. So we corrected the deformity with a forehead free flap and the result was cosmetically acceptable. There was no evidence of recurrence or distant metastasis for one- year follow- up period. CONCLUSIONS: Despite its high recurrence rate, we can reduce the recurrence and mortality rate of squamous cell carcinoma of the lacrimal sac with radical excision including bony and mucous NLD, inferior turbinate, and medial maxilla.