To present the proportion of patients with periocular basal cell carcinoma (BCC) who underwent orbital exenteration and to evaluate the significance of the following risk factors: initial tumor site, pathologic features, and initial treatment.
Retrospective, comparative, interventional case series.
Charts of all patients with BCC referred to Orbital Unit of the University of Naples “Federico II” between 1984 and 2003 were reviewed. Charts were reviewed for patient demographics, previous treatments, tumor site, clinical presentation, duration of symptoms, and histologic subtype. The main outcomes were recurrence rate, tumor-related deaths, orbital infiltration, and rate of exenteration.
Data (including follow-up) were available for 506 patients. Twenty-eight patients (5.5%) underwent orbital exenteration. For 8 patients (28.5%), orbital exenteration was the first procedure performed. In the exenterated group, the most common tumor site was the medial cantus, whereas in the overall group, it was the lower eyelid ( P = .001). The proportion of patients initially treated without margin control was significantly higher in patients undergoing exenteration ( P = .0001). Pathologic examination revealed a higher incidence of infiltrative subtype in the exenterated group ( P = .00019).
The need for exenteration for BCC may be significantly higher when the lesion involves a medial canthal location, initial management does not include margin-controlled excision, or pathologic analysis reveals an infiltrative subtype. Margin-controlled excision for periocular BCC and close follow-up after excision for medial canthal BCC may be indicated.
Basal cell carcinoma (BCC) is the most common malignant periocular tumor, accounting for 90% of malignant eyelid lesions. It occurs most commonly in older, fair-skinned individuals, and it is associated with ultraviolet exposure. The most frequent periocular site of BCC is the lower eyelid, followed by the medial canthus, the upper eyelid, and the lateral canthus. Although mortality from BBC of the eyelid is very low, the associated morbidity can be significant. The goal of treatment is complete removal of the tumor to prevent recurrence. The incidence of orbital invasion is approximately 2% to 4%, and risk factors include multiple recurrences, large lesion size, aggressive histologic subtype, medial canthal location, and advanced patient age. In this retrospective study, we sought to identify the proportion of patients with periocular BCC requiring exenteration and to describe risk factors associated with the need for this radical surgery.
Charts of all patients with periocular BCC referred to the Orbital Unit of the University of Naples “Federico II” between January 1984 and December 2003 were reviewed. The diagnosis of BCC was confirmed by pathologic analysis in all patients. Patient demographics, previous treatments, tumor site, clinical presentation, duration of symptoms, and histologic subtype were analyzed and compared between all patients with BCC and those requiring exenteration to determine their significance as risk factors for orbital exenteration. Surgical procedures for local excision were performed by different surgeons (D.S., A.I., G.U., G.B.) using the following standard procedure. Clinically clear margins were marked around the tumor by noting the transition in surface contour, vascularization, skin color, and surface texture. The extent of tumor depth was judged by the macroscopic features of the excision plane, and margins of 3 mm were marked with the skin under tension. Fast-track paraffin section margin control was used in all patients treated with local excision. Reconstruction was performed immediately using direct closure when possible and was carried out with flaps or grafts after the results on margins were known. Exenteration was performed in patients with clinical and radiologic evidence of orbital invasion. The outcome measures were recurrence rates, tumor-related deaths, orbital infiltration, and rate of exenteration. Institutional review board approval was obtained, and each patient gave informed consent. Associations between categorical variables were analyzed using the chi-square test. All analyses were performed using MedCalc software version 11.2 (MedCalc Software, Mariakerke, Belgium).
Data, including follow-up, were available for all 506 patients (52% male) identified during the defined study period. The mean patient age was 67.2 years (range, 47 to 84 years). All patients were white. The mean follow-up period was 3.2 years (range, 0.3 to 15 years). A total of 28 patients (5.5%) underwent orbital exenteration. Of these 28 patients, 15 were males (53.6%) and 13 were females (46.4%), with a mean age of 68.4 years (range, 46 to 84 years).
Before exenteration, 20 patients (71.4%) underwent 1 or more surgical excisions for BCC. For 16 patients (80%), initial therapy was provided elsewhere, whereas 4 patients (20%) received initial treatment at the study institution. The mean time from the first excision to the diagnosis of orbital invasion was 92 months (range, 6 months to 30 years). The average number of recurrences requiring surgical excision was 3.6 (range, 3 to 10). The remaining 8 patients (28.5%) had not undergone any surgery before exenteration. Six patients had extensive disease involving the orbit at presentation. In 1 patient, the previous treating physician had underestimated the extent of the tumor, and the remaining patient had been treated with external radiotherapy without excisional biopsy.
All of the patients requiring orbital exenteration had a visible or palpable mass. The most common signs of orbital invasion were bone fixation of the mass (40.6%), followed by limited ocular motility (31.3%), and globe displacement (18.7%). For 10 patients (31.3%), only a visible or palpable mass was noted, with no evident orbital involvement. Computed tomography imaging data were available for 26 patients, and orbital extension was apparent in 23 patients (82%). Signs of bone involvement were evident on computed tomography scans in 8 patients (28.5%).
Among all 506 patients, the most common periocular tumor site was the lower eyelid (58.1%), followed by the medial canthus (25.1%). The right and left side of the face were affected almost equally (49.6% and 50.4%, respectively). In the 28 patients who underwent exenteration, the most common initial tumor site was the medial canthus (53.6%), followed by the lower eyelid (35.7%), the upper eyelid (7.1%), and the lateral canthus (3.6%; P = .0007).
The distribution of histologic subtypes among the 28 patients undergoing exenteration was as follows: infiltrative (78.6%), basosquamous (14.2%), morpheaform or sclerosing (3.6%), and nodular (3.6%). This distribution of histologic subtypes in these 28 patients was significantly different compared with the histologic distribution for remaining 478 patients with periocular BCC ( P = .0001). Initial margin involvement data were available for 26 patients; in 19 patients (73%), the margins were not clear ( Table 1 ).
|Exenterated Patients (n = 28), N (%)||Nonexenterated Patients (n = 478), N (%)||P Value a|
|Tumor site: medial canthus||15 (53.6)||106 (22.2)||.0007|
|Tumor site: lower eyelid||10 (35.7)||288 (60.3)||.02|
|Histologic subtype: infiltrative||22 (78.6)||170 (35.5)||< .0001|
|Histologic subtype: nodular||1 (3.6)||266 (55.6)||< .0001|
Twenty patients (71.4%) underwent exenteration after 1 or several conservative surgical excisions. In 8 patients (28.6%), exenteration was performed as the first surgical treatment because of the evident orbital invasion at the time of initial presentation; 2 of these patients (7.2%) underwent postoperative radiotherapy. In 14 patients (50%), orbital exenteration was carried out after 1 or more surgical excisions. Six patients (21.4%) underwent surgical excision and radiotherapy before exenteration ( Table 2 ).
|Treatment||No. of Patients (n = 28)|
|Exenteration alone||6 (21.4%)|
|Exenteration + radiotherapy||2 (7.2%)|
|Surgical excision + exenteration||14 (50%)|
|Excision + radiotherapy + exenteration||6 (21.4%)|
In the 20 patients who underwent exenteration after other surgical excisions, 16 (80%) showed no margin control. In contrast, of the 478 nonexenterated patients who underwent 1 or more surgical excisions, 80 (16.7%) had uncontrolled margins ( P < .0001, between-group comparisons).
Postoperative orbital margin data were available for 24 of the 28 patients who underwent exenteration. In 12 patients (50%) the margins were clear of tumor. However, in the remaining 12 patients, the margins were not clear, and 7 of these patients were treated with adjuvant radiotherapy to control the disease. Four patients declined further surgical procedures.
Postexenteration recurrence was diagnosed in 8 patients (28.5%); 7 (87.5%) had positive margins at exenteration, and 4 had undergone radiotherapy. In these patients, further surgical excision was necessary. Of the 28 exenterated patients, 2 (7.1%) died as a result of BCC, both as a result of intracranial invasion. Both patients had a history of recurrent tumor, had a medial canthal infiltrative BCC, and had undergone radiotherapy after orbital exenteration.