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Setting
We present a second case from The Lion Heart Medical Centre in Yele, Sierra Leone. This new small rural hospital has a capacity of thirty-four beds. It has a functioning operation theatre, a small laboratory and digital radiography equipment. The nearest specialist hospitals are six hours away by car.
| Case report A forty-five year old man presented at the outpatient ward with an ulcerating swelling on the occipital side of his head. Three years before, the lesion had appeared as a small nodule that erupted spontaneously with evacuation of pus. Since two years the nodule gradually increased in size and became ulcerated. The ulcer had been treated with native herbs, without effect. Because of the growing discomfort, the patient decided to consult a doctor. He had always been in good health. Besides discomfort caused by the ulcer and headache, there were no complaints. There were no history of trauma, no systemic signs of infection, no loss of weight, nor signs of neurological impairment. There were no other relatives with similar complaints. On examination, there were no abnormalities besides the lesion on the skull. There was a circular ulcerating wound of seven by seven centimetres on the frontoparietal side of the skull. The lesion looked infected and showed partial healing. The edges of the wound were raised and were firm on palpation. The base of the lesion was soft and there was a depression of the underlying skull. (Figure 1) There was no lymphadenopathy. An X-ray of the skull showed destruction and infiltration of the frontoparietal cortex. (Figure 2) A malignancy such as a squamous cell carcinoma or a basal cell carcinoma was suspected. To treat possible secondary osteomyelitis an oral course of cloxacillin was started. To exclude any other possible underlying diseases Consult Online was asked for advice about differential diagnosis and further treatment. |
Advice from the specialists
Two dermatologists and three surgeons replied within two days.
The suggestion of a malignancy was supported. It was mentioned that both squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) are less frequently seen in dark skinned individuals. Of both forms, SCC occurs more frequently in this population. However, it was also felt that after three years of disease, SSC would probably have shown more progression and might have disseminated.
Further suggested differential diagnoses were tuberculosis and other slowly destructive fungal, bacterial or parasitic infections. A chronic osteomyelitis starting from a superficial infection was also mentioned as an option. In addition, as an alternative option, it was proposed there could also have been a not mentioned trauma with a skull fracture and a meningeal tear, through which oedematose brain tissue could protrude.
To demonstrate fungal infection, it was advised to perform a potassium hydroxide (KOH) smear.
One of the specialists mentioned personal experience with using pulp from leaves of the periwinkle Catharanthus roseus (old name Vinca rosea) for palliative local treatment of non-resectable ulcerating breast carcinoma in Ghana. Vinca alkaloids such as vinblastine, vincristine, vindesine, vinorelbine, vinflunine and anhydrovinblastine, inhibit tubulin assembly and therefore have an anti-mitotic effect. These compounds are used as chemotherapy for various forms of cancer. [1]
Palliative treatment with Vinca leaves or empirical treatment with anti-tuberculous drugs could be considered. Because surgery in these situations is very difficult, wearing of a protective helmet was advised.
Course
Following the advice, a chest X-ray was done, which showed no signs of tuberculosis. Empirical treatment with anti-tuberculous drugs could not be given due to a shortage of drugs at the time of consultation. A course of oral griseofulvin was started to treat a possible fungal infection.
A biopsy of the lesion was taken and sent to the Netherlands for pathological examination. After two weeks the results of the histology confirmed a squamous cell carcinoma. The case was discussed with a dermatologist member of a Dutch head and neck oncology team. The advice in a Dutch situation would have been to resect the lesion with a margin of 2 cm. If the tumour is restricted to the tabula externa of the skull bone, the tabula interna would not have to be resected. However, if the tabula interna is also affected, the tumour should be completely removed. To close the wound a reconstruction with prosthetic material would be necessary. Postoperative radiotherapy would also be required. If the tumour has invaded the brain, neurosurgery or palliative chemoradiotherapy should be considered.
In Sierra Leone, chemotherapy or radiotherapy is hardly available and neurosurgery is not available at all. On X-ray both tabula interna and externa seemed to be affected. Therefore, it would be necessary to perform a complicated surgical procedure with the risk of meningeal tear and secondary infection. In addition, there is no prosthetic material available in the hospital. The patient was counselled with regard to all these issues and he was advised to start palliative treatment with painkillers, treatment of secondary infection and wearing a protective helmet.
Discussion
Primary cutaneous SCC is a malignant epithelial tumour. Worldwide incidence rates of SCC vary depending on whether SCC is counted together with premalignant conditions. SCC and BCC are both among the most common forms of human cancer. [2]
The key factor for developing SCC is exposure to ultraviolet radiation (UVR). People with a poor capacity for tanning are more at risk. Chronic inflammation, chronic wounds and immunosuppression are additional risk factors for developing SCC. [2,3] Actinic Keratosis and Bowen’s disease are premalignant conditions of SCC. [2]
In dark skinned individuals, SCC is more frequent than BCC and may be unrelated to UVR, as it is also found in covered parts of the skin. In addition, in black populations SCC frequently arises in parts of the skin with chronic inflammation. [3]
There is a wide range of treatment options and the decision depends on prognostic classification of primary SCC (‘low risk’ or ‘high risk’) and metastatic stage. Clearly, prevention is of the utmost importance. [2]
Conclusion
In this case report we presented a patient with an invasive cutaneous SCC of the skull with unknown metastatic stage. This is the first published case report of Consult Online in which biopsy material was analysed in the Netherlands, which could lead to very specific treatment recommendations. Unfortunately, due to the restrictions of the low-resource setting and the complexity of the case, operation and chemoradiotherapy are not possible. Palliative treatment is the only available option. Nonetheless, a firm diagnosis was made and that enabled the specialists to adequately counsel the patient. Therefore, other empirical and potentially harmful treatment was avoided.
This case illustrates the risk of chronic exposure to UVR, even in dark skinned populations. However, preventative measures other than wearing a hat and clothes while working in the sun, are probably not practical in developing countries. Because SCC is also found in covered parts of the skin and tends to start in sites with chronic infection, there is also the need for early diagnosis and treatment of chronic skin infections.
References
- Kingston DGI. Tubulin-Interactive Natural Products as Anticancer Agents. J Nat Prod. 2009;72:507-15.
- Bonerandi JJ, Beauvillain C, Caquant L, Chassagne JF, Chaussade V, Clavère P, et al. Guidelines for the diagnosis and treatment of cutaneous squamous cell carcinoma and precursor lesions. JEADV. 2011;25:1-51.
- Lucas R, McMichael T, Smith W, Armstrong B. Solar ultraviolet radiation. Global burden of disease from solar ultraviolet radiation. Environmental Burden of Disease Series, No. 13. Geneva: World Health Organisation; 2006.
