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1 MUCINOUS ADENOCARCINOMAS OF NOSE AND PARANASAL SINUSES.I. J. GAMEZ-ARAUJO, MD,* ALBERTO G. AYALA, OSCAR GUILLAMONDEGUI, MD MD, AND This study concerns the Clinicopathologic findings for 18 patients with mucinous adenocarcinomas of nose and/or paranasal sinuses. Males in the 5th decade of life predominated in the series. Nasal obstruction, a growing mass in a sinus, or epistaxis were the most frequent complaints. Ten patients had tumors in the maxillary antrum, and the nasal cavity was the site in 5 patients. Histopathologically, the tumors were papillary or solid. The majority of the papillary tumors were to well, with a few poorly types. Tumors with the solid pattern of growth were anaplastic; these patients had poorer prognoses. For most patients, treatment consisted of radical surgery alone or in combination with radiotherapy. Of 13 patients for whom survival could be adequately evaluated, 7 died from the tumors, 5 are alive and free of disease more than 4 years, and 1 is living with recurrent tumor 14 months after diagnosis. Cancer 36:llOO-1105, ALICNANT NEOPLASMS OF THE NOSE AND M paranasal sinuses comprise approximately 15% of cancers of the upper respiratory tract. lo Squamous carcinoma is the malignancy most frequently encountered, representing 80-90% of patients. Adenocarcinomas are rare; they have been estimated to represent 4-8% of carcinomas of the nose and paranasal sinuses. This report concerns the clinicopathologic findings in 18 patients with mucinous adenocarcinomas of the nose and/or paranasal sinuses treated at The University of Texas System Cancer Center, hl. D. Anderson Hospital and Tumor Institute, from 1952 to CLINICAL FINDINGS The patients ages varied from 25 to 80 years. The majority (15) were in the 5th, 6th, or 7th decades. I he mean age was 59.6 years. The From l he University of Texas System Cancer Center, hl. D. Anderson Hospital and Tumor Institute, Houston, 1.X * Senior Fellow in Pathology. Assistant Pathologist, Assistant Professor of Pathology. I Assistant Professor of, Assistant Surgeon. Head and Neck Section. Address for reprints: i\lberto G. Ayala, MD, Dept. of Pathology. Al. I). Anderson Hospital and Tumor Institute, Texas hledical Center, Houston, TX The authors thank 31rs. hlary hlccrackan for editing and Xlrs. Jeanne Knight for secretarial assistance. Received lor publication.july 30, group included 17 males and 1 female; 14 patients were Caucasian and 4 were Negro. The most frequent symptoms were nasal obstruction, a growing mass in a paranasal sinus, and epistaxis. In 10 patients the tumor was located in the maxillary antrum (6 in the left and 4 in the right). The nasal cavity was the next most frequent location (5 patients). The extent of the disease was determined by radiologic examination, according to the Baclesse method or a modification of the Sisson method Those lesions in the anterior, lateral, or anterior portion of the medial walls of the maxillary sinus were considered infrastructural. The ones in the remaining portion of the medial wall, the posterior and superior walls of the maxillary sinus, and the ethmoidal and sphenoidal sinus were of suprastructure location. In 14 patients, the neoplasms were of the suprastructural nature. In the remaining 4 the lesions were of an infrastructural nature. The treatment consisted of surgical resection alone in 8 patients. This procedure entailed partial or total maxillectomy combined with orbit exenteration. An additional 7 patients received postoperative irradiation therapy. Irradiation therapy was the only modality of treatment for 2 patients; 1 other patient was treated with a combination of irradiation and chemotherapy (Table 1). Local recurrence of the tumor was observed in 1100

2 No. 3 ADENOCARCINOMA OF NOSE AND PARANASAL SINUSES Gamez-Araujo el al TABLE 1, Mucinous Adenocarcinomas of Nose and Paranasal Sinuses Current starus* Age Race/ Recur- hletas- (survival Case (yr) sex Location Histology Treatment rence tasis in months) C/M Left middle turbinate Papillary well C/hl Right nostril Solid Radiation NED (2000 rads) + surgery Radiation DUD (4000 rads) 72 C/F Left antrum Solid poorly 1 Regional cervical 66 C/Xl Right inferior turbinate Papillary well n 0 135NED 61 C/Xl Right antrum Papillary n 0 implants 14 DSU 70 N/hl Left nostril Papillary (6000 rads) 1 Regional 69 DT cervical lymph nodes 65 N/Xl Left antrum Papillary x I)T C/hl Left antrum Papillary poorly X2 Regional cervical 35 DT lymph nodes C/Il Right antrum Solid poorly Palliative X: 1 Brain. right 2 DT surgery neck, right (autopsy) excision nodes and lungs C/XI Right sphenoid and ethmoid sinuses Solid poorly Radiation (6000 rads) + chemotherapy 0 n 9 DSU 56 C/hI Right sphenoid sinuses Papillary poorly Radiation (3000 rads) XI 10 DT 80 N/hl Right antrum Solid poorly (6200 rads) n 2 DT 60 (:/XI Left ethmoid Papillav moderate I y XO 58 I)SU 42 C/Xl Left antrum Papillary Surge? (6500 rads) x2 40 NED 63 C/Sl Left antrum Papillary well 0 I DU1) 41 C/Xl Left antrum Papillary well x3 132NEI)

3 1102 CANCER September 1975 Vol. 36 Table 1 (continued) Current status. :\qe Race/ Recur- hlctas- (survival (:are (yr) sex Location Histology Treat men1 rencc tasis in months) (17) BI) S9 N/h4 Right antrum Solid poorly Radiation NED (5000 rads) + surgery (18) BH 54 C/hl Left middle turbinate Papillary well x NEI) (6100rads) * NED = no evidence of disease; DUD = dead, unrelated disease; DT = Dead of tumor; DSLI = disease status unknown. nine patients, six of whom developed recurrences within 12 months after the initial treatment. Metastases to regional cervical lymph nodes developed in four patients, behavior similar to that of squamous carcinoma of the paranasal sinuses. In one individual, the tumor invaded the brain, and also metastasized to the lungs. Kadiographic studies demonstrated local bone destruction in 15 patients. Only 13 patients in this study can be adequately evaluated in relation to survival. Seven of these 13 patients died as a consequence of the tumor. Six other patients were alive and free of disease; 1 of them for 40 months, another patient for 48 months, and 4 for more than 5 years following diagnosis. The average length of survival for patients who died of their disease was 22.5 months. One patient was living with recurrent tumor 14 months after the diagnosis (Table 1 ). Inadequate followup necessitated elimination of 5 patients. Patient 2 and 15 died of unrelated disease 1 month after diagnosis. Patient 5 died 14 months and Patient 10, 9 months after diagnosis, but status of disease could not be determined at that time. Patient 13 survived 58 months, experienced nine recurrences, but was lost to followup 11 months before his death. HISTOPATHOLOGIC FINDINGS Gross In 12 of the patients who underwent surgical resections, gross specimens were available for Ftc. 1. Papillary adenocarcinoma. The tumor nodules consist of multiple papillary projections and a central cystic space containing necrotic material and cellular debris. These nodules are surrounded by thick bands of connective tissue (X32).

4 No. 3 ADENOCARCINOMA OF NOSE AND PARANASAL SINUSES Garnet-Araujo et al review. The size of the neoplasms varied from 2.5 to 6 cm, for an average of 5 cm. Two were polypoid, 2 others were described as cauliflower-like tumors. The remaining 8 lesions were solid and poorly circumscribed. None displayed capsules. The color varied from gray to white or dark brownish. Necrosis and hemorrhage were grossly evident in 10 tumors, and in 1 the cut surface was described as mucoid. Microscopic A papillary pattern was seen in 12 tumors; 6 were of solid type. The papillary tumors displayed a range between a well- gland arrangement to an anaplastic, frankly malignant epithelium, which often projected into cystic spaces containing mucin or cellular debris (Fig. 1). Five tumors were well, 6 were well, and 1 was poorly. The cells were generally cuboidal to cylindrical with sharp cytoplasmic boundaries, varying amounts of eosinophilic to amphophylic cytoplasm and hyperpigmented irregular nuclei. Abnormal mitoses were not infrequent. Mucin was found in all tumors; 8 showed rather abundant production. In 1 tumor mucin was so prominent it resembled the colloid type of carcinoma of the gastrointestinal tract. The remaining six tumors showing the solid pattern were poorly and composed of sheets or cords of irregularly sized and shaped cells, which occasionally formed glandlike spaces. The cytoplasm was variable in amount, and the nuclei demonstrated hyperchromatism and prominent nucleoli. Three of these tumors deserve special description. They showed diffuse sheets of large round cells with abundant cytoplasm. The nuclei occasionally were pushed aside, giving a signet-ring cell appearance (Fig. 2). In other areas the tumors simulated clear cell neoplasms such as renal cell or squamous carcinoma, but were distinguished by demonstration of mucin production, using the Alcian-blue method (Fig. 3). Bone involvement was observed in all but 2 of the 18 patients. Metastases to regional cervical lymph nodes were proved in 4 patients. DISCUSSION hlucinous adenocarcinomas of nose and paranasal sinuses probably arise from glandular epithelium of the surface or from tissue having a common origin with that of gastrointestinal tra~t. ~ The majority of these neoplasms histologically resemble adenocarcinomas of the large bowel. 8*13.14 In the series of 18 patients reported here, tumors were more frequent in men, and in the 5th decade of life. This is in accord with findings Fit;. 2. Signet-ring cell rype of adenocarcinoma (X400).

5 1104 CANCER September 1975 Vol. 36 FIG. 3. Carcinoma, "solid" type. This tumor shows diffuse sheets of medium to large size cells with abundant clear cytoplasm and defined cytoplasmic borders. Delicate bands of connective tissue with capillaries give support to this tumor. of other authors.'." Many articles in the English literature agree that workers in the footwear repairing, wood, and furniture industries have increased risks of developing nasal and paranasal adenocarcinoma. '*' No valid conclusions can be drawn from the small series presented here regarding the role of occupation in the development of this neoplasm. The location of the neoplasm usually determines the type of symptomatology. All the tumors in the nose produced nasal obstruction. Adenocarcinomas from the maxillary antrum usually manifested as a growing mass, and less frequently with epistaxis or rhinorrhea. Although bat saki^,^ Kingertz," and Jarvi' have reported the nasal cavity and ethmoid sinuses as the most frequent sites, in the present series the antrum and the nasal cavity were the predominant locations. At the time of diagnosis most of these neoplasms had already extended to adjacent anatomical structures. This circumstance frequently precludes an accurate evaluation of the exact site of origin. Kingertz," who thoroughly studied the pathology of the tumors of nose and paranasal sinuses grouped the adenocarcinomas into "alveolar" or "pseudopapillary. " In the current study, the tumors were simply subclassified as "papillary" or "solid" according to the predominant pattern. The grading of these cases according to cellular atypia and degree of differentiation appeared to correlate with survival. Except for Case 17, all patients with poorly tumors died within 16 months after the diagnosis. The rarity of distant metastasis has been stressed by Kingertz, I' Jarvi,' Sanchez, l3 and Simard and Jean. I' The morphological pattern of metastasis in the nodes may resemble that of gastrointestinal cancer. This fact must be emphasized in order to avoid an erroneous interpretation and fruitless search for a different primary lesion. In regard to adenocarcinomas of the clear cell type displaying a "solid" pattern, pathologists should be alerted to the possibility of a lesion metastatic from a renal cell carcinoma. Special stains to demonstrate mucin production are mandatory in such cases. The frequency of metastasis to the head from renal carcinoma remains controversial. Batsakis' and Bernstein' referred to renal carcinoma as the tumor most frequently giving rise to metastasis to the nose and paranasal sinuses. J ~rtay,~ in a review of 40 cases, found no metastatic lesions from the kidney. Surgical resection combined with pre- or postoperative irradiation therapy is the treatment of choice for adenocarcinoma of the nose and paranasal sinuses. The best results are ob-

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