Primary Lacrimal Sac Tumors with Extension into Vicinity: Outcomes with Endoscopy-Assisted Modified Weber-Ferguson's Approach.

PURPOSE: To evaluate the outcomes of endoscopy-assisted modified Weber-Ferguson's approach in the management of primary lacrimal sac tumors with extension into the neighboring tissues. METHODS: A retrospective interventional study was performed on all patients with lacrimal sac tumors treated with the endoscopy-assisted modified Weber-Ferguson approach between January 2010 and June 2022 at the Shanghai Ninth People's Hospital, China. Data assessed include demographics, clinical presentations, imaging features, surgical techniques, histopathology, adjuvant modalities of management, complications, and outcomes. RESULTS: A total of 13 patients were included in the analysis. Epiphora and palpable mass lesion were the presenting complaint in 84.6% (11/13) of the patients. Nearly half of the patients (46.1%, 6/13) were misdiagnosed as lacrimal duct obstruction. All the lacrimal sac tumors in the present series showed uneven enhancement on T1-weighted MRI imaging. Postoperatively, 84.6% (11/13) patients recovered well with excellent esthetics and were disease-free after a mean follow-up of 58.6 months. Two patients who underwent additional exenteration developed recurrence and succumbed (at 41 and 96 months follow up) while they were on palliative chemoradiation. CONCLUSION: The endoscopic-assisted modified Weber-Fergusson surgical approach is effective in providing better visibility and accessibility to lacrimal sac tumors with extension into neighboring tissue.

HER2-Positive Lacrimal Sac Squamous Cell Carcinoma in a 57-Year-Old Man.

Introduction: Lacrimal sac squamous cell carcinoma (SCC) is a rare tumor. Only 241 cases of lacrimal sac SCC have been reported in the literature. However, the detailed molecular profile of this tumor is unknown. Case Presentation: Fifty-seven-year-old Caucasian male presented with a 6-month history of epiphora. Multimodal examination revealed a unilateral lacrimal sac SCC T4aN0M0. The patient underwent primary surgery with subsequent chemoradiotherapy. The patient was alive 18 months after the end of the treatment, with no signs of local or distant relapse. Complex molecular profiling revealed the FGFR p.G388R variant, HER2 amplification, and progression phenotype. Conclusion: Here, we describe a clinical case of a male patient with lacrimal sac SCC with a careful description of the disease history, treatment, and molecular-genetic patterns of the tumor. This is the first report of HER2-positive lacrimal sac SCC.

Lacrimal Sac Tumors: A Single-institution Experience, Including New Insights.

BACKGROUND/AIM: Lacrimal sac tumors are rare tumor types, with a long time interval from disease onset to diagnosis. We aimed to investigate the characteristics and outcomes of patients with lacrimal sac tumors. PATIENTS AND METHODS: The medical records of 25 patients with lacrimal sac tumors initially treated at the Kyushu university hospital from January 1996 to July 2020 were reviewed. RESULTS: Our analysis included 3 epithelial benign tumors (12.0%) and 22 malignant (88.0%) tumors (squamous cell carcinoma, n=6; adenoid cystic carcinoma, n=2; sebaceous adenocarcinoma, n=2; mucoepidermoid carcinoma, n=1; malignant lymphoma, n=10). The average time from symptom onset to diagnosis was 14.7 months (median=8 months; range=1-96 months). The analysis of patients revealed that lacrimal sac mass (22/25, 88.0%) was the most frequent symptom and a possible tumor marker. Most epithelial benign (n=3) and malignant epithelial (n=12) tumors were treated surgically (14/15, 93.3%). One malignant case was treated with heavy ion beam therapy. Eight patients were treated with postoperative (chemo)radiation therapy because of positive surgical margins (including one unanalyzed case). Local control was ultimately achieved in all but one case. The patient survived for 24 months with immune checkpoint inhibitors and subsequent chemotherapy for local and metastatic recurrence. CONCLUSION: We report our experience in the diagnosis and treatment of lacrimal sac tumors and analyze the clinical trends in cases involving these tumors. Postoperative radiotherapy and pharmacotherapy, including immune checkpoint inhibitors, may be useful for recurrent cases.

Acquired nasolacrimal duct obstruction: clinical and histological findings of 275 cases.

BACKGROUND: Acquired nasolacrimal duct obstruction is a blockage of the lacrimal outflow system usually caused by local nonspecific inflammation of the lacrimal sac and the nasolacrimal duct. However, cases exist where the primary nasolacrimal system obstruction is caused by malignancies. Our aim was to investigate lacrimal sac pathologies in patients with acquired nasolacrimal duct obstruction and compare their clinical manifestations. METHODS: This retrospective clinical study included 275 patients with acquired nasolacrimal duct obstruction who underwent external dacryocystorhinostomy and lacrimal sac biopsy. Cases were classified into tumor or nonspecific pathology groups and subdivided according to the level of inflammation. Histological and clinical data were analyzed. RESULTS: Three tumors (1.1%) (an adenoid cystic carcinoma, an eccrine spiradenoma and small B cell lymphoma) were diagnosed. Chronic nongranulomatous inflammation was the most common histological finding, corresponding to 194 cases (70.5%). The other 81 (29.5%) were subacute, acute forms of nongranulomatous inflammation, tumors and fibrosis cases. Epiphora with continuous purulent discharge was the most common clinical sign reported by 144 (52.4%) patients, and two (0.7%) patients had a palpable mass near the medial canthal tendon, which was identified as an eccrine spiradenoma and small B cell lymphoma. There was no significant difference in the clinical symptoms, duration or case history between the nonspecific pathology and tumor groups (p = 0.292). CONCLUSIONS: Chronic nongranulomatous inflammation of the lacrimal sac was the most common finding among acquired nasolacrimal duct obstruction cases. There were no associations between the histological findings and clinical presentation. The authors recommend a lacrimal sac biopsy only in cases when a tumor is clinically suspected.

Orbital involvement in lacrimal drainage disorders.

Lacrimal sac is situated anterior to the orbital septum, which acts as a barrier, thus limiting the posterior migration of the pathologies affecting the lacrimal drainage system. Certain pathologies can breach this barrier and secondarily involve the orbit causing significant clinical manifestations. This posterior migration of pathology also has a significant influence on the management and outcomes. The present paper will discuss the lacrimal pathologies which secondarily involve the orbit and its influence on the management and outcomes.

Epiphora and unrecognized paranasal sinuses pathology.

PURPOSE: to report five patients all presenting with persistent unilateral epiphora as a sign of unexpected and rare lesions causing Secondary Acquired Nasolacrimal Duct Obstruction (SANDO) and the risks associated to an incomplete diagnostic work-up. OBSERVATIONS: the cases presented are: (1) Fungus ball, (2) Pyogenic granuloma, (3) Sinonasal inverted papilloma (4) Sinonasal inverted papilloma with synchronous squamous cell carcinoma, (5) Squamous cell carcinoma of the lacrimal sac. CONCLUSIONS AND IMPORTANCE: masses are uncommon but not a rare cause of nasolacrimal duct obstruction. Surgical teams performing large numbers of dacryocystorhinostomies should be aware of such pathology and perform a systematic multidisciplinary approach.

Valor del ultrasonido en el diagnóstico de los tumores del saco lagrimal / Value of the ultrasound in the diagnosis of lacrimal sac tumor

RESUMEN Los tumores del saco lagrimal son extremadamente raros, y el benigno primario más común es el papiloma escamoso. Clínicamente se manifiestan como una zona de tumefacción en la región cantal medial, asociado a epifora y dacriocistitis crónica-recurrente. Dentro de las pruebas de imagen recomendadas están: dacriocistografía, tomografía computarizada, resonancia magnética y dacrioescintifotografía. Sin embargo, muy pocos sugieren el uso del ultrasonido. Se presenta el caso de un papiloma mixto del saco lagrimal diagnosticado por ultrasonido. Se trata de una paciente femenina de 39 años, quien fue remitida por cuadros repetidos de dacriocistitis derecha en los últimos 3 años. En consulta se constató celulitis preseptal ipsolateral, por lo que se indicó antibiótico-terapia, y se logró la remisión de los signos inflamatorios agudos, pero persistió el aumento del volumen cantal medial derecho. La vía lagrimal se exploró y se comprobó que estaba obstruida. Se realizó ultrasonido, donde el saco lagrimal derecho apareció ocupado por una masa ecogénica, que se extendía al conducto lacrimonasal y mostraba ligera vascularidad. Seguidamente se hizo tomografía computarizada, y se confirmaron los hallazgos ecográficos, pero sin lograr delimitar la lesión intrasaco descrita, aún tras la administración de contraste yodado endovenoso. Se sugirió dacriocistitis crónica derecha, secundaria al tumor intrasaco de aspecto benigno, probable papiloma, por lo que se efectuó cirugía y examen histopatológico que ratificaron dicho diagnóstico. En conclusión, el ultrasonido del saco lagrimal es un método complementario de gran valor, que en algunos casos hará el diagnóstico, y en otros brindará información que optimizará la decisión de quienes serían tributarios a estudios más complejos(AU)

ABSTRACT Lacrimal sac tumors are extremely rare, and the most common benign primary is squamous papilloma. Clinically, they manifest as a zone of swelling in the medial canthal region, associated with epiphora and chronic-recurrent dacryocystitis. Among the recommended imaging tests are dacryostography, computed tomography, magnetic resonance imaging, and dacryoscintifotography. However, very few suggest the use of ultrasound. The case of a mixed papilloma of the lacrimal sac diagnosed by ultrasound is presented. This is a 39-year-old female patient, who was referred for repeated cases of right dacryocystitis in the last three years. In consultation, ipsolateral preseptal cellulitis was found, for which antibiotic therapy was indicated, and the remission of acute inflammatory signs was achieved, but the increase in right medial canthal volume persisted. The lacrimal duct was explored and found to be obstructed. Ultrasound was performed, where the right lacrimal sac appeared occupied by an echogenic mass, which extended to the lacrimal duct and showed slight vascularity. Subsequently, computed tomography was performed, and the ultrasound findings were confirmed, but without defining the described intrasaccal lesion, even after administration of intravenous iodinated contrast. It was suggested chronic right dacryocystitis, secondary to apparently benign intrasaccal tumor, probable papilloma, so surgery and histopathological examination were performed, which that ratified the diagnosis. In conclusion, ultrasound of the lacrimal sac is a complementary method of great value, which in some cases will make the diagnosis, while in others it will provide information that will optimize the decision of those who would be candidate to more complex studies(AU)

An Unusual Presentation of Large B-cell Lymphoma.

Primary Non-Hodgkin's lymphoma of the lacrimal sac is extremely rare. Symptoms are usually atypical and nonspecific, which often leads to the original misdiagnosis of dacryocystitis. The most common presenting features are epiphora, swelling, and acute dacryocystitis. We present a case of a 67-year-old female with primary diffuse large B-cell lymphoma (DLBCL) of the lacrimal sac, which was originally diagnosed as dacryocystitis. This case report adds to the urgency that prompt and precise diagnosis and treatment is key.

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.

Lacrimal Sac Tumors--A Review.

Tumors of the lacrimal sac are rare but their recognition and early management are imperative, as they are locally invasive and potentially life-threatening. Because of their rarity, large clinical studies with statistically significant data on the natural course, management, and prognosis of these neoplasms are unavailable. Current practices are therefore based on a few case series and a small number of isolated case reports. Most tumors are primary and of epithelial origin (60-94%), of which 55% are malignant. Lacrimal sac tumors typically present with epiphora and a palpable mass over the medial canthus and are thus often erroneously diagnosed as chronic dacryocystitis. A full history with clinical and diagnostic workup is essential to plan treatment, which is often multi-disciplinary. Statistically significant associations have been shown with higher tumor staging and size with increased metastatic risk and lower survival rates. Management usually involves complete surgical resection with adjuvant radiotherapy and/or chemotherapy for malignant lesions. Long-term follow-up is required, as recurrences and metastases can occur many years after initial treatment.