|Year : 2021 | Volume
| Issue : 4 | Page : 731-735
Solitary fibrous tumor of the lacrimal sac: A rare entity and a brief review of the literature
Sahil Agrawal1, Aishwarya Rathod1, Sujeeth Modaboyina1, Seema Sen2, Deepsekhar Das1, Mandeep S Bajaj1
1 Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
2 Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||28-Jan-2021|
|Date of Acceptance||07-Apr-2021|
|Date of Web Publication||09-Oct-2021|
Dr. Deepsekhar Das
Oculoplasty and Orbital Tumor Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm of mesenchymal fibroblast-like cells. A 47-year-old female, with complaints of watering with left medial canthal fullness and left failed dacryocystorhinostomy, on imaging showed homogeneous well-defined lesion extending into nasolacrimal duct with adjacent bone remodeling. Excisional biopsy showed spindle-cell tumor with cellular areas showing prominent vascular pattern. On immunohistochemistry, CD-34 was positive and Ki-67 was <5%. A diagnosis of SFT was suggested. At 6 months follow-up, the patient had no recurrences.
Keywords: Lacrimal sac tumor, rare nasal tumor, sinonasal tract tumor, solitary fibrous tumor, spindle cell tumor
|How to cite this article:|
Agrawal S, Rathod A, Modaboyina S, Sen S, Das D, Bajaj MS. Solitary fibrous tumor of the lacrimal sac: A rare entity and a brief review of the literature. Indian J Ophthalmol Case Rep 2021;1:731-5
|How to cite this URL:|
Agrawal S, Rathod A, Modaboyina S, Sen S, Das D, Bajaj MS. Solitary fibrous tumor of the lacrimal sac: A rare entity and a brief review of the literature. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:731-5. Available from: https://www.ijoreports.in/text.asp?2021/1/4/731/327628
Solitary fibrous tumors (SFTs) are rare benign spindle-cell neoplasms that arise from serosal surfaces such as pleura and peritoneum. It can also uncommonly occur in the lacrimal sac and have features of orbital, sinonasal, or both the components. Immunohistochemistry plays a vital role in their diagnosis as they have heterogeneous clinical and histological appearances and are very rarely seen. Here, we report a case of this rare tumor and compare our findings with the other 11 available cases in the literature.
| Case Report|| |
A 47-year-old female presented to the ophthalmology outpatient department with complaints of watering along with medial canthal fullness of the left eye for 5 years [Figure 1]a. The swelling was gradually progressive and painless. She gave a history of left-sided dacryocystorhinostomy, which was done 10 years back.
|Figure 1: (a) Clinical picture of the patient showing a mass in the left medial canthus causing medial canthal dystopia. (b) Clinical picture of the patient at 6 months follow-up showing normalized left medial canthus. (c) Contrast-enhanced computed tomography of the head and orbit, coronal cut, showing a homogeneous well-defined lesion in the left lacrimal sac area. (d) Gross specimen of the lacrimal sac tumor after en bloc excision of the mass|
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On general examination, she was alert and conscious with stable vitals. On ophthalmic examination, the vision in both eyes was 6/6 with accurate projection of rays in all quadrants, and intraocular pressure (IOP) was within normal limits. There was swelling involving the left lacrimal sac area extending from the bridge of nose to the medial canthus. There was associated medial canthal dystopia, with the left medial canthus being pushed upward and laterally. On palpation, the swelling was firm, nontender, and nonreducible with a smooth surface. It was not fixed to the overlying skin. There was no regurgitation on pressure over the lacrimal sac and no evidence of any discharge. Anterior segment and posterior segments were within normal limits. Probing and syringing of the left lacrimal system revealed obstruction.
On contrast-enhanced computed tomography (CECT) of the head and orbit, a homogeneous well-defined lesion 3.3 cm × 1.8 cm was noted in the lacrimal sac area, which was extending into the nasolacrimal duct with adjacent bone remodeling [Figure 1]c.
A clinical diagnosis of lacrimal sac tumor was made, and she was planned for a dacryocystectomy surgery. En bloc excision of the mass, lacrimal sac along with the nasolacrimal duct, was performed through external approach, and the tissue was sent for histopathological examination [Figure 1]d. On gross examination, the tumor measured 3.5 cm × 2 cm, and on light microscopy, it showed a spindle-cell tumor with variable cellularity and areas of collagenization [Figure 2]a and normal lacrimal sac lining of stratified columnar epithelium with goblet cells overlying the tumor [Figure 2]b. The cellular areas showed a prominent vascular pattern [Figure 2]c. There was no cytological atypia or mitosis seen. Immunohistochemically, CD-34 showed strong positivity [Figure 2]d. Immunostains for S-100 and cytokeratin were negative. Ki-67 was <5%. Based on these features, a diagnosis of SFT was made. At 6 months follow-up, the patient was doing well without any evidence of recurrence [Figure 1]b.
|Figure 2: (a) Light microscopy shows a spindle-cell tumor with areas of low cellularity and collagenization (H and E 200×). (b) Normal lacrimal sac lining of stratified columnar epithelium with goblet cells overlying the tumor. (c) Cellular area with prominent vascular pattern and no cytological atypia (H and E 200×). (d) CD-34 cytoplasmic positivity in tumor cells (Avidin Biotin 200×)|
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| Discussion|| |
SFT is a rare spindle-cell tumor arising from the mesenchyme. It was first described in 1931 by Klemperer and Rabin as a distinct mesothelial tumor arising from the pleura. Later on many cases have been described that can present at extrapleural sites, that is, subcutaneous tissues, orbit, lacrimal sac, paranasal sinuses, nasal cavity, meninges, extremities, thoracic wall, and abdomen (liver, adrenal, peritoneum, urogenital system). Lesions earlier referred to as “hemangiopericytoma” of soft tissue are probably best classified as SFT.
The most common extracranial head and neck sites were sinonasal tract and orbit involving about 25% of head and neck SFTs. Dorfman et al. and Westra et al. described orbital involvement for the first time in 1994. Orbital involvement is rare, and in our literature review we were able to find only 64 cases till date. The major factor of etiopathogenesis is NAB2-STAT6 gene fusions, secondary to intrachromosomal rearrangements (translocations) on chromosome 12q13.
Immunohistochemistry classically shows positivity for CD-34, CD-99, and STAT6 nuclear staining. One-third cases are also positive for EMA, BCL2, and actin. The condition is usually benign but can undergo malignant transformation. Features of malignancy include mitotic figures >4/10 HPF (high-power field) and size >10 cm. In cases of malignant transformation, it has been found to metastasize via the hematogenous route most commonly to the lungs. Lymphatic spread is exceedingly rare.
On conducting a thorough literature search, we could find only 11 cases of SFT of lacrimal sac reported till date, two of which were from the Indian subcontinent. This is the third case to be reported from the Indian subcontinent [Table 1]. It usually presents as watering, nasolacrimal duct obstruction, and medial canthal swelling that is painless and gradually increasing in size or recurrent dacryocystitis. Although not pathognomonic, high vascularity due to prominent vascular channels with the tumor make them homogeneous- or heterogeneous-attenuated enhance.
|Table 1: Previously reported cases of solitary fibrous tumor of the lacrimal sac|
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Histopathological examination of an SFT shows a spindle-cell morphology with prominent vascularity with thick bands of collagen and no features of malignancy such as tumor necrosis, cellular atypia, and so on. However, as SFTs have diverse histopathological features, it can be confused with other tumors such as mesotheliomas and sarcomas., Immunohistochemistry is, therefore, vital and confirmatory with CD-34 and vimentin positivity and S-100 and desmin negativity.,,
Complete surgical excision of the mass is a must. Partial resection can lead to recurrence or malignant transformation. SFTs are relatively chemoresistant. Early stages of the disease confined within the lacrimal sac have a good prognosis, whereas tumors with extension, malignant transformation, and large size have poorer prognosis. Because incomplete excision can lead to recurrence or malignant transformation, long-term follow-up is to be done for these patients.
This case report adhered to the ethical principles outlined in the Declaration of Helsinki as amended in 2013.
| Conclusion|| |
SFT of the lacrimal sac is extremely rare. It should be one of the differential diagnosis for a patient with nasolacrimal duct obstruction with a painless firm mass in the lacrimal sac.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]