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Facial extensive ulcer

Úlcera extensa facial

SUMMARY

Basosquamous carcinoma (BSC), as described in 1910, is a distinctive variety of skin cancer and its etiology and pathological characteristics have generated much controversy over the years. Currently, BSC is considered a basal cell carcinoma (BCC) subtype with aggressive behavior and greater tendency for recurrence and metastases. We present a clinical case recently reported in our unit.

Keywords:
skin neoplasms; skin ulcer

RESUMO

Carcinoma basoescamoso, conforme descrito em 1910, é uma variedade distinta de câncer de pele e sua etiologia e características patológicas têm gerado muita controvérsia ao longo dos anos. Atualmente, BSC é considerado um subtipo de carcinoma de células basais com comportamento agressivo e maior tendência para a recorrência e metástase. Nós apresentamos um caso clínico relatado recentemente em nossa unidade.

Palavras-chave:
dermatopatias; neoplasias cutâneas

Basosquamous carcinoma (BSC), as described in 1910, is a distinctive variety of skin cancer and its etiology and pathological characteristics have generated much controversy over the years.11 Volkenstein S, Wohlschlaeger J, Liebau J, Arens A, Lehnerdt G, Jahnke K, Neumann A. Basosquamous carcinoma- a rare but aggressive skin malignancy. J Plast Reconstr Aesthet Surg 2010; 63: e304-6. Currently, BSC is considered a basal cell carcinoma (BCC) subtype with aggressive behavior and greater tendency for recurrence and metastases.

CASE

A 52-year-old male presented with a 6-year history of an impressive extensive ulcer on his right cheek (Figure 1). It appeared over the old scar of a previous BCC excised ten years ago. It had painless steady increase in size with progressive difficulty in ocular movements and epiphora. A facial CT scan demonstrated a destruction of the right maxilla and nasal bone with no lymphadenopathies in the head and neck regions. Blood cell counts, urea, sedimentation rate, electrolytes, urinalysis tests were within reference ranges and antinuclear and anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Mycological and bacterial cultures were also negative. A skin biopsy was performed and sent for hematoxylin-eosin and immunohistological staining (Figure 2).

FIGURE 1
A 6-year slow growing facial ulcer developed over an old scar.
FIGURE 2
Infiltrative basal cell carcinoma (left side) adjacent to a squamous differentiation area with neoplastic cells containing eosinophilic cytoplasm and central keratinization (right side) (A,B,C). Positive immunostaining with Ber-EP4 in basaloid areas, contrasting negative Ber-EP4 in squamous differentiation area (D).

DISCUSSION

The incidence of BSC is between 1.5-2.7% in the largest studies reported.22 Martin R, Edwards MJ, Cawte TG, Sewell CL, McMasters KM. Basosquamous carcinoma: analysis of prognostic factors influencing recurrence. Cancer 2000; 88: 1365-9. Local aggressive growth pattern and high potential for distant metastases are the main concerning facts. The rate of local recurrence of the BSC is 45%, almost twice as much as squamous cell carcinoma (SCC) and BCC.33 Bowman PH, Ratz JL, Knoepp TG, Barnes CJ, Finley EM. Basosquamous carcinoma. Dermatol Surg 2003; 29: 830-2. This rare skin carcinoma has a metastatic rate of 5-8.4%. Prognostic factors for recurrence include positive resection margins, lymphatic invasion, perineural invasion and male gender.44 Costantino D, Lowe L, Brown DL. Basosquamous carcinoma-an under-recognized, high-risk cutaneous neoplasm: case study and review of the literature. J Plast Reconstr Aesthet Surg 2006; 59: 424-8.

Clinical image of the lesion is non-specific but long- -standing time of evolution is a common feature in all the patients reported. The majority of these tumors arise on the head and the neck (80%), the central face and perinasal areas being the most prevalent locations.55 Betti R, Crosti C, Ghiozzi S, Cerri A, Moneghini L, Menni S. Basosquamous cell carcinoma: a survey of 76 patients and a comparative analysis of basal cell carcinomas and squamous cell carcinomas. Eur J Dermatol 2013; 23: 83-6.

Histological examination exhibits findings of both BCC and SCC with a transition zone. It is not clear if BSC develops de novo or evolves from a pre-existing lesion, but the "squamatization theory" is the most commonly accepted. Unfortunately, the patient's previous BCC that was excised 10 years before could not be re-evaluated. Immunohistochemical stains have helped to a better characterization of BSC. Areas of BCC are Ber-EP4, AE1 and AE3 positive. In contrast, CAM5.2 and a variable positivization of epithelial membrane antigen are identified in the SCC areas. Ber-EP4 stain is gradually negative in the transition zone.66 Garcia C, Poletti E, Crowson AN. Basosquamous carcinoma. J Am Acad Dermatol. 2009; 60: 137-43.

Non-prospective trials to compare the different therapeutic regimes to approach BSC are available. There are also no specific consensus management guidelines reported in the literature. Due to its aggressive local behavior pattern, Mohs micrographic surgery is recommended and should be standardized as it occurs in other skin tumors such as dermatofibrosarcoma protuberans.77 Veronese F, Farinelli P, Zavattaro E, Zuccoli R, Bonvini D, Leigheb G, Colombo E. Basal cell carcinoma of the head region: therapeutical results of 350 lesions treated with Mohs micrographic surgery. J Eur Acad Dermatol Venereol 2012; 26: 838-43.Local radiation is a fitting alternative in selected cases. Curettage and fulguration or cryotherapy should not be considered unless the patient refuses an invasive surgical procedure. Vismodegib is the first selective inhibitor of the Hedgehog signaling pathway to be approved for the treatment of locally advanced and metastatic BCC. It could add a different therapeutic approach in advanced BSC; however, there is no case reported to date. The introduction of vismodegib, an orally active inhibitor of the Hedgehog signaling pathway by binding to Smoothened, approved for the treatment of locally advanced and metastatic BCC could add a different therapeutic approach in advanced BSC. There is no case reported to date.

In patients with localized disease, metastasis to the regional lymph nodes is the strongest predictor of recurrence and survival. Selective sentinel node biopsy should be considered even in absence of palpable lymphadenopathies in order to manage a proper staging of this entity.88 Jankovic I, Kovacevic P, Visnjic M, Jankovic D, Binic I, Jankovic A, Ilic I. Application of sentinel lymph node biopsy in cutaneous basosquamous carcinoma. Ann Dermatol 2011; 23: S123-6.

In conclusion, we suggest that all dermatologists should be aware that BSC may not be focused in the same terms as BCC and SCC. Individualized guidelines should be employed.

  • Study conducted at the Hospital Alto Guadalquivir, Andujar, Jaen and at the Hospital Universitario Virgen de las Nieves, Granada, Spain

REFERENCES

  • 1
    Volkenstein S, Wohlschlaeger J, Liebau J, Arens A, Lehnerdt G, Jahnke K, Neumann A. Basosquamous carcinoma- a rare but aggressive skin malignancy. J Plast Reconstr Aesthet Surg 2010; 63: e304-6.
  • 2
    Martin R, Edwards MJ, Cawte TG, Sewell CL, McMasters KM. Basosquamous carcinoma: analysis of prognostic factors influencing recurrence. Cancer 2000; 88: 1365-9.
  • 3
    Bowman PH, Ratz JL, Knoepp TG, Barnes CJ, Finley EM. Basosquamous carcinoma. Dermatol Surg 2003; 29: 830-2.
  • 4
    Costantino D, Lowe L, Brown DL. Basosquamous carcinoma-an under-recognized, high-risk cutaneous neoplasm: case study and review of the literature. J Plast Reconstr Aesthet Surg 2006; 59: 424-8.
  • 5
    Betti R, Crosti C, Ghiozzi S, Cerri A, Moneghini L, Menni S. Basosquamous cell carcinoma: a survey of 76 patients and a comparative analysis of basal cell carcinomas and squamous cell carcinomas. Eur J Dermatol 2013; 23: 83-6.
  • 6
    Garcia C, Poletti E, Crowson AN. Basosquamous carcinoma. J Am Acad Dermatol. 2009; 60: 137-43.
  • 7
    Veronese F, Farinelli P, Zavattaro E, Zuccoli R, Bonvini D, Leigheb G, Colombo E. Basal cell carcinoma of the head region: therapeutical results of 350 lesions treated with Mohs micrographic surgery. J Eur Acad Dermatol Venereol 2012; 26: 838-43.
  • 8
    Jankovic I, Kovacevic P, Visnjic M, Jankovic D, Binic I, Jankovic A, Ilic I. Application of sentinel lymph node biopsy in cutaneous basosquamous carcinoma. Ann Dermatol 2011; 23: S123-6.

Publication Dates

  • Publication in this collection
    Jan-Feb 2016

History

  • Received
    30 Jan 2015
  • Accepted
    17 Feb 2015
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