A 72-year-old woman, in good general performance status, with 4-year symptoms of a slowly growing tumor located at the base of the nose, is presented. The first symptom of disease was a small tubercle located at the base of the nose, near to the internal angle of the right eye, which occurred in January 2003. The patient was initially diagnosed by an ophthalmologist in Rzeszow, Poland, and computed tomography (CT) was ordered. It was performed in May 20, 2003 and it revealed: "Within the sclerotic ethmoid sinus, osteolytic defect with diameter 2 cm and Hounsfield density 37 is present, filled by soft-tissue mass. Penetration to frontal sinus and right orbit with destruction of its medial wall is observed but without infiltration of intraorbital structures".
A representative sample for histopathology examination was taken and showed: "Abundant inflammatory infiltration composed of plasmatic and lymphocytic cells, epithelial granulomas with caseation necrosis, tuberculous like, focally pyonecrosis and squamous cell metaplasia within surface" (Department of Pathology, Rzeszow). Based on this, the decision to leave the patient in regular follow-up was taken.
One month later control CT showed stable disease, with suggestion of sarcoidosis and tuberculosis as well as the possibility of Wegener's granulomatosis. Chest X-ray and abdominal cavity ultrasonography excluded the presence of other pathologies. All blood tests (morphology, liver and renal parameters) and urinalysis were in the normal ranges, so follow-up examinations were suggested.
The next CT, performed on September 13, 2003 revealed: "At the right base of nose, soft tissue mass with size 3.1 x 2.9 cm, non-enhancing significantly after contrast injection, is observed. This pathologic structure brings skin in frontal region into relief, penetrates into right orbit with pressure on its structure, resulting in exophthalmos. Tumor is located in non-formed right frontal sinus. It causes massive destruction of right frontal bone, nasal bones, especially right, laminae of the ethmoid bone. Tumor does not penetrate into anterior cranial fossa. Destruction of nasal conchae (upper and middle), nasal septum and medial walls of both maxillary sinuses causing communication with nasal cavity is present. Progression of disease".
As a consequence of disease progression, verification of histopathology specimens was performed in the Department of Pathology, Cancer Center and Institute of Oncology in Warsaw (October 2003). A diagnosis of Wegener's granulomatosis was ascertained. Immunosuppression therapy was started: between October 2003 and March 2004 therapy with cyclophosphamide and prednisone was applied, and then therapy based on methotrexate and prednisone was continued to January 2005. Partial regression with long lasting (more than two years) stagnation of tumor size was observed.
In June 2007 the patient reported the regrowth of the tumor. For this, radiotherapy was offered and the patient was directed to the Cancer Center and Institute of Oncology, Gliwice Branch.
The first visit and the examination in the First Department of Radiation Oncology of the Institute took place on September 21, 2007. Frontal headache and excessive right eye tear secretion were the main complaints of the patient.
During physical examination a smooth, well-contoured tumor located at the right base of the nose and near the internal angle of the right eye with size 5 x 4 cm (measured with skin, skin not infiltrated) was described. The tumor caused a slight right exophthalmos, but without eyeball movement limitation, diplopia or vision defects. CT performed in our institute was consistent with the previous one, but progression to 3.4 cm in the greatest dimension was observed (Figs. 1A, 1B).
Radiotherapy was performed in November, 12-29, 2007 in the First Department of Radiation Oncology. The treatment was planned with the intensity modulated radiotherapy (IMRT) technique. The arrangement of the irradiation fields is presented in Fig. 2. Because of close proximity to the right eyeball and lens, a total radiation dose of 30 Gy conventionally fractionated (2 Gy) was prescribed. Therefore the maximal radiation dose within the right lens did not exceed 5 Gy. No acute effects were observed; tolerance of treatment was very good. The left lens received a radiation dose insignificant for late complications.
Routine follow-up has been performed since the end of treatment in our center. No regression was observed during the first visit, two months after radiotherapy. However, slow, but gradual shrinkage progressed, at first as flattening of the tumor and next as decrease of its dimensions. The regression process lasted 12 months, to March 2009, when complete clinical response was achieved. To ascertain radiological remission, in June 29, 2009, computerized tomography was done - "At present CT no previously described tumor is observed. In its bed only scarring and loss of tissue without contrast enhancement is present. Also partial loss in nasal and frontal bones is seen. Part of nasal septum, nasal conchae and middle walls of maxillary sinuses are not visualized" (Figs. 3A, 3B).
The eye examination, performed 3 years after the end of radiotherapy, showed no radiation complications within the organ of vision. Also acuity of vision was still in the normal range.
The last examination was performed in March 2011 and no symptoms of relapse were observed.