Not all basal cell carcinomas are created equal: A case of a fatal BCC
Department of Dermatology, University of Oklahoma, Oklahoma City, Oklahoma
Basal cell carcinoma is one of the most common malignancies in the world. It is usually a low-grade malignancy, but may invade deeper structures and metastasize in less than 0.1 percent of cases. Giant basal cell carcinoma is a rare, much more aggressive subtype greater than 5 cm in diameter. In the article, the authors present a case of a neglected giant basal cell carcinoma that resulted in death and provide a brief update on the literature.
Basal cell carcinoma (BCC) is the most common skin cancer in the United States. Giant basal cell carcinoma (GBCC) is a subtype greater than 5 cm that accounts for 1 percent of cases. In this article, the authors present a case of a fatal GBCC.
A 62-year-old male presented to his primary care doctor with a chief complaint of a painful, enlarging “spider bite” on his back. He denied a history of fever, chills, weight loss, or night sweats. The patient had no significant past medical history, last seeing a physician over 10 years ago. On physical examination, a 19 cm x 19 cm ulcerated plaque was noted, extending from the patient’s thoracic to lumbar back. The ulceration penetrated 6 cm, exposing vertebrae (Figure 1). A skin biopsy was performed, revealing infiltrating, atypical, basaloid cells (Figures 2 through 4). An MRI of the spine revealed a large enhancing soft tissue mass extending from the 9th thoracic vertebra to the 3rd lumbar vertebra with invasion of the 10th and 11th thoracic vertebrae as well as the left posterior 10th rib. A CT at presentation showed spiculated lung nodules and a hypoattenuated liver lesion. A fine needle aspiration of one lung lesion was non-diagnostic for malignancy.
A diagnosis of infiltrating giant basal cell carcinoma was established. The tumor was treated with neoadjuvant radiation therapy (74 Gy total) that resulted in a 75 percent reduction in size. At oncology follow-up, the patient was considered a poor candidate for surgery or chemotherapy because of a persistently poor nutritional status (prealbumin 9.5 mg/dl), ECOG performance status of 3, and new imaging studies revealing a concern for metastases. Treatment options were then discussed, and the patient chose to be placed on palliative care. Thereafter, the patient died 18 months after initial diagnosis. BCC metastasis was believed to be the cause of death. However it could not be definitively determined because the patient had no further workup after being placed on palliative care and the family deferred an autopsy.
Basal cell carcinoma is most often found on sun-exposed regions in adults as an ulcerated or pearly papule, plaque, or nodule . The pathogenesis of BCC involves mutations in the sonic hedgehog pathway and p53 gene . Risk factors include light skin color, UV exposure, radiation, certain hereditary diseases including Gorlin syndrome, and immunosuppression . Basal cell carcinoma is usually a low-grade malignancy. However BCCs may invade deeper structures including the spinal cord and may metastasize in less than 0.1 percent of cases, most commonly to lung, bone, and lymph nodes . Higher risk BCCs include size greater than 2 cm, location in H-zone of face, recurrence, and aggressive subtype . Histological subtypes include superficial, nodular, micronodular, pigmented, sclerosing, infiltrating, and basosqaumous. Histology most often shows atypical basaloid cells with peripheral pallisading surrounded by stromal retraction artifact . Current treatment options include electrodessication and curettage, topical fluorouracil or imiquimod, photodynamic therapy, cryotherapy, regular excision, Mohs surgery, chemotherapy (methotrexate, bleomycin, vincristine, 5-FU, platinum, and taxanes), and radiation. Vismodegib, a hedgehog pathway inhibitor, is now available for metastatic BCCs and those not treatable with radiation or surgery [2, 4]. This medication would have been indicated in our patient if commercially available at the time of diagnosis. A detailed algorithm for BCC treatment can be found on the NCCN’s website .
Giant basal cell carcinoma is an aggressive subtype of BCC greater than 5 cm in diameter, accounting for approximately 1 percent of BCC cases. It is most often found in men in their 7th decade on the face and back; it is most often attributed to neglect. The most common histological subtypes are nodular (53%) and infiltrating (20%). These cancers are usually treated with surgery using 1 cm margins or Mohs micrographic surgery. Non-surgical treatments include those discussed above . Giant basal cell carcinomas are much more likely to metastasize with reported rates of 45 percent in BCCs > 10 cm and 100 percent in BCCs > 25 cm in diameter [6, 7]. Median survival after metastasis is 8-14 months . The overall prognosis of GBCC is poor with a reported cure rate of 61.7 percent by a mean 2-year follow-up .
Giant basal cell carcinoma is a dangerous subtype of BCC with a much poorer prognosis. Although many of these cancers are curable, they may sometimes result in death. In our patient, the major limiting factors for survival included late presentation and the patient’s poor overall health at presentation. As discussed by Zoccali et al, these lesions must be diagnosed and treated early to achieve the best outcomes .
References1. Thompson LD. Skin basal cell carcinoma. Ear Nose Throat J. 2010 Sep;89(9):418-20. [PubMed]]
2. Kwasniak LA, Garcia-Zuazaga J. Basal cell carcinoma: evidence-based medicine and review of treatment modalities. Int J Dermatol. 2011 Jun;50(6):645-58. Review. [PubMed
3. Cohen B, Weiss G, Yin H. Basal cell carcinoma (BCC) causing spinal cord compression. Dermatol Online J. 2000 Sep;6(1):12. [PubMed]
4. Von Hoff DD et al. Inhibition of the hedgehog pathway in advanced basal-cell carcinoma. N Engl J Med. 2009 Sep 17;361(12):1164-72. Epub 2009 Sep 2. [PubMed]
5. Zoccali G et al. Giant basal cell carcinoma of the skin: literature review and personal experience. J Eur Acad Dermatol Venereol. 2011 Dec 28. [PubMed]
6. Sahl WJ Jr, Snow SN, Levine NS. Giant basal cell carcinoma. Report of two cases and review of the literature. J Am Acad Dermatol. 1994 May;30(5 Pt 2):856-9. [PubMed]
7. Snow SN et al. Metastatic basal cell carcinoma. Report of five cases. Cancer. 1994 Jan 15;73(2):328-35. [PubMed]
8. Ting PT, Kasper R, Arlette JP. Metastatic basal cell carcinoma: report of two cases and literature review. J Cutan Med Surg. 2005 Jan;9(1):10-5. [PubMed]
9. Archontaki M et al. Giant Basal cell carcinoma: clinicopathological analysis of 51 cases and review of the literature. Anticancer Res. 2009 Jul;29(7):2655-63. [PubMed]
© 2013 Dermatology Online Journal