Correspondence Address:
Dr. Sinai Khandeparkar Siddhi Gaurish
E-517, The Island, Wakad, Pune - 411 057, Maharashtra
India

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Editor,

A 50 years old female presented to ear nose throat out-patient department with complaints of the right nasal block and epistaxis since 15 days. Local examination showed a reddish fleshy mass protruding out of the right nostril [Figure 1]a. She was a known case of diabetes mellitus and hypothyroidism on treatment since 5 years. She tested seronegative for HIV. Computed tomography of the paranasal sinuses (PNS) done showed the presence of soft tissue density, mildly enhancing lesion in the right posterior nasal choana extending into the nasopharynx [Figure 1]b, bilateral ethmoidal, and sphenoid sinus. There was an erosion of the upper part of the nasal septum, ethmoidal trabeculae, medial wall of the right maxillary sinus, and focal erosion in the lamina papyracea on the right side. Magnetic resonance imaging of the PNS done in addition showed an intracranial component measuring 3.2 cm × 3.4 cm × 3.9 cm in the basifrontal region. With a provisional diagnosis of neoplastic lesion, the mass was excised.{Figure 1}

On gross examination, multiple brownish white, friable, and few polypoidal soft tissue bits were received aggregating to 8 cc [Figure 1]c. Microscopically, polypoid bits were composed of necro-inflammatory tissue. At places, sheets of tumor cells were seen lining the anastomosing vascular channels [Figure 1]d. They were composed of cells with hyperchromatic, pleomorphic and at places vesicular nuclei with prominent nucleoli, and a moderate amount of cytoplasm. The tumor exhibited high mitotic activity (10–12 mitosis/high power field). Few red blood cells were seen within the lacunae formed by rudimentary lumen formation by the tumor cells [[Figure 1]d inset]. Areas of hemorrhage and necrosis were noted. Diagnosis of high grade angiosarcoma was considered.

Immunohistochemistry (IHC) was performed with the following panel of antibodies viz. Pan CK, vimentin, epithelial membrane antigen (EMA), desmin, smooth muscle actin (SMA), CD31, and Ki-67. The tumor cells showed immunoreactivity for vimentin [Figure 2]a, EMA, and CD31 [Figure 2]b. They were nonimmunoreactive for Pan CK, desmin, and SMA. Ki-67 labeling index (KLI) was 40% [Figure 2]c. The diagnosis of high grade angiosarcoma was confirmed. The patient expired in a month's time before receiving chemoradiotherapy.{Figure 2}

Angiosarcomas comprise <1% of all sarcomas.[1] Primary sinonasal tract angiosarcomas (SNA) account for <0.1% of all sinonasal tract malignancies.[2] The rarity of the tumor causes its frequent misclassification and thus inappropriate management. Accurate diagnosis is based on immuno-histopathological evaluation, which is indispensable not only for determining tumor type but also for prognostication. Most SNA documented are histologically low grade.[3] We encountered this rare case of aggressive poorly differentiated SNA, highlighting the diagnostic significance of IHC markers.

SNA shows male predilection with a peak incidence in the fifth decade. Females tend to be younger at presentation by up to a decade.[3] Our case was a 50-year-old female. The patients tend to have symptoms for an average of 9.8 months.[4] Our patient had symptoms for a very brief period of 15 days depicting its aggressive nature.

It has been documented that, there is better prognosis for SNA when compared to their skin, soft tissue, or visceral counterparts. This is attributed to the earlier stage at diagnosis because of epistaxis as a presenting symptom which results in an earlier tumor detection.[1] The authors believe that in this case possibly there was antecedent involvement of PNS, subsequent polypoidal mass leading to nasal obstruction, and epistaxis leading to advanced lesion at presentation.

Within the sinonasal tract, a single anatomic site is more commonly affected than multiple sites. Maxillary sinus is most commonly affected.[4] It is important to note that the lesion in present case was extending to multiple sites.

The differential diagnosis includes granulation tissue, intravascular papillary endothelial hyperplasia, lobular capillary hemangioma, juvenile nasopharyngeal angiofibroma, epithelioid hemangioma, glomangiopericytoma, Kaposi's sarcoma, and malignant melanoma.[5]

Immunohistochemically, the tumor cells show immunoreactivity with vimentin, CD34, CD31, and factor VIII-RA, while focally reactive with keratin or EMA and SMA. The KLI of >10% suggests a high proliferative activity. CD31 is a highly specific marker and is expressed in 90% of cases. A panel approach allows for a greater degree of certainty.[4]

Complete surgical excision is the treatment of choice in well-delineated and solitary tumors. Early accurate diagnosis along with surgical excision, chemo-radiotherapy is of benefit in multifocal, ill-defined tumors.[3]

Although very few SNA have been reported, our experience with this case highlights the need for high index of suspicion, significance of IHC markers for arriving at accurate diagnosis, and the fact that some of the cases may be very high grade lesions at presentation.

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.

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Nil.

Conflicts of interest

There are no conflicts of interest.

References