Epistaxis is a common complaint encountered in clinical practice and usually ceases spontaneously. However, certain recurrent intractable cases caused by several rare diseases can cause considerable morbidity. A systematic approach is required to establish the diagnosis in such conditions. We report a case of a 52-year-old man who presented with chronic recurrent Epistaxis due to polymorphous low-grade Adenocarcinoma (PLGA). This is the rare case of chronic Epistaxis caused by PLGA. Complete examination and timely surgery are the key components of a successful treatment strategy for such rare disorders.

Wang TC, Yang YT, Tsai MT (2016) Polymorphous Low-Grade Adenocarcinoma: A Rare Cause of Long-Term Epistaxis. J Ear Nose Throat Disord 1(1): 1002.

•    Epistaxis
•    Nasal polyp
•    Polymorphous low-grade adenocarcinoma

PLGA: Polymorphous Low-Grade Adenocarcinoma

Epistaxis is a common problem that affects approximately 60% of all people in their lifetime [1] and is usually a benign self-limiting illness. However, it can occasionally be intractable and is caused by several rare diseases. Refractory epistaxisis attributable to hypertension, anti platelet medications, and alcohol abuse [2]; however, most cases are typically idiopathic. A systematic approach is required to establish the diagnosis of Epistaxis in such conditions. In this report, we present a case in which polymorphous low-gradeadenocarcinoma (PLGA), a rare disorder of the sinonasal tract, caused chronic recurrent epistaxis in an adult male and discuss management strategies for these lesions.

A 52-year-old man presented with a 30-month history of intermittent Epistaxis and purulent discharge from the left naris. He was under regular aspirin medication for preventing coronary artery disease. In addition, he had undergone bilateral Caldwell–Luc operation for benign maxillary sinus disease 15 years previously. He visited different clinics several times for the aforementioned symptoms, which were subsequently diagnosed as chronic paranasal sinusitis and the adverse effects of antiplatelet agents. Aspirin was discontinued; however, it did not reduce the frequency and severity of the nose bleeds. Therefore, he visited our department to seek medical help. On physical examination, no obvious lesions or bleeders were observed over the common meatus, septum, or vestibule. Further evaluation through fiber optic endoscopy revealed scant crust accumulation in the left middle meatus, and an easily bleeding and nonfriable lobulated mass with neovascularization was identified after removal of the crust (Figure 1a). The extent of the mass was evaluated through contrast-enhanced computed tomography, which revealed soft tissue enhancement in the left ethmoid sinus and nasal cavity. No intra orbital destruction or skull base invasion was observed (Figure 1b). The left lamina papyracea was unremarkable.

Biopsy reported basal cell adenoma, but malignant carcinoma cannot be ruled out. After discussing with patient, modified endoscopic medial maxillectomies were performed 2 weeks later under general anesthesia. The dissection plane was directed toward the skull base and lateral to the periorbita. The structures above this region were intact, and no gross residual tumor was observed. The excised tumor specimen measured 3.5 cm × 1.2 cm × 0.7 cm. The microscopy of the sections revealed PLGA comprising irregular infiltrating solid nests and cuboidal cells growing in an adenoid cystic pattern arranged in ducts, trabeculae, and tubules (Figure 2a). Under high magnification, most tumor cells were bland and relatively uniform. The round and ovoid nuclei were normal in size or slightly enlarged, and lymph vascular invasion was observed (Figure 2b). Mitoses were infrequent. Perineural, perivascular, and bony invasion, involving nearly all portions of the obtained fragments, were observed.

The pathologic staging was pT2Nx, and the patient received radiation therapy with a daily dose of 200 cGY and a total dose of up to 7000 cGY. In addition, tegafur/uracil at 300 mg/day in divided doses was administered as a post operative adjuvant therapy for 6 months. Epistaxis did not recur after surgery. Follow-up magnetic resonance imaging and fiberoptic endoscopy 25 months after treatment revealed tumor-free nasosinal cavities with mild scarring (Figure 3).

Epistaxis in Sino-nasal tract tumor can be fatal due to the massive size of the tumor leading to outstripping its blood supply. But in PLGA, the bleeding is often scanty rather than massive by its less aggressive entity. As in this case, the histopathology finding doesn’t exhibit obvious hemorrhagic infarction and necrosis of the tumor. These characters may explain why epistaxis is intermittent and doesn’t lead to severe constitutional symptoms for such a long time.

PLGA is a minor salivary gland neoplasm that arises mostly in the oral cavity, particularly in the mucosa of the soft and hard palates 3. PLGA presenting as a primary tumor of the extra oral region is rare, and only a few cases of PLGA in the sinonasal location have been reported [4,5]. These tumors tend to present as sinonasal polyps with or without nasal obstruction. Less common symptoms include pain, bleeding, and ulceration.

PLGA may exhibit a combination of several distinct growth patterns: tubular, trabecular, cribriform, and solid. In contrast to the morphological diversity, the nuclei are typically uniform with inconspicuous mitotic activity[6]. PLGA can usually be diagnosed from the typical cytological and histological features.

PLGA frequently exhibits an infiltrative growth pattern, lymphovascular, and perineural invasion; however, the prognosis for such patients is often favorable. The tumor is usually indolent, slow-growing, locally invasive, and rarely metastatic [7]. Wide local excision with appropriate margins is the current first-line treatment for the majority of PLGA [3]. Routine neck dissection is not recommended unless clinical evidence supporting lymph node involvement is available. The benefits of adjuvant therapy for PLGA remain controversial. Studies have reported that postoperative radiation therapy has a survival benefit for PLGA arising at unusual locations, such as the parotid glands [8]. The present case was successfully treated through surgery and adjuvant chemotherapy with postoperative radiation therapy; no recurrence was observed 25 months after the treatment.

In conclusion, PLGA is a rare cause of chronic intractable epistaxis and can be easily ignored. A fiberscopic examination is mandatory in such condition for definite diagnosis, and prompt surgery can usually improve the outcome. Additional studies are warranted to verify the role of adjuvant radiation therapy and chemotherapy in these tumors.

1. Petruson B, Rudin R. The frequency of epistaxis in a male population sample. Rhinology. 1975; 13: 129-133.

2. Jackson KR, Jackson RT. Factors associated with active, refractory epistaxis. Arch Otolaryngol Head Neck Surg. 1988; 114: 862-865.

3. Evans HL, Batsakis JG. Polymorphous low-grade adenocarcinoma of minor salivary glands. A study of 14 cases of a distinctive neoplasm. Cancer. 1984; 53: 935-942.

4. Wenig BM, Harpaz N, DelBridge C. Polymorphous low-grade adenocarcinoma of seromucous glands of the nasopharynx. A report of a case and a discussion of the morphologic and immuno histochemical features. Am J Clin Pathol. 1989; 92: 104-109.

5. Lengyel E, Somogyi A, Gõdény M, Szerdahelyi A, Németh G. Polymorphous low-grade adenocarcinoma of the nasopharynx. Case report and review of the literature. Strahlenther Onkol. 2000; 176: 40-42.

6. McHugh JB, Visscher DW, Barnes EL. Update on selected salivary gland neoplasms. Arch Pathol Lab Med. 2009; 133: 1763-1774.

7. Castle JT, Thompson LD, Frommelt RA, Wenig BM, Kessler HP. Polymorphous low grade adenocarcinoma: a clinicopathologic study of 164 cases. Cancer. 1999; 86: 207-219.

8. Uemaetomari I, Tabuchi K, Tobita T, Tsuji S, Wada T, Kamma H, et al. The importance of postoperative radiotherapy against polymorphous low-grade adenocarcinoma of the parotid gland: case report and review of the literature. Tohoku J Exp Med. 2007; 211: 297-302.