|LETTER TO EDITOR
|Year : 2009 | Volume
| Issue : 1 | Page : 72-73
Rare occurrence of carcinoma esophagus in a case of epidermolysis bullosa
A Ray, S Bhattacharya, A Kumar, K Bhattacharya
Department of Radiation Oncology, Advanced Medicare Research Institute, Dhakuria, Kolkata, India
Department of Radiation Oncology, Advanced Medicare Research Institute, Dhakuria, Kolkata
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Ray A, Bhattacharya S, Kumar A, Bhattacharya K. Rare occurrence of carcinoma esophagus in a case of epidermolysis bullosa. Indian J Cancer 2009;46:72-3
|How to cite this URL:|
Ray A, Bhattacharya S, Kumar A, Bhattacharya K. Rare occurrence of carcinoma esophagus in a case of epidermolysis bullosa. Indian J Cancer [serial online] 2009 [cited 2020 Jul 4];46:72-3. Available from: http://www.indianjcancer.com/text.asp?2009/46/1/72/48602
Epidermolysis bullosa (EB) is a rare genetic disease associated with muco-cutaneous blistering and extremely fragile skin. It presents in one of three forms-EB simplex, junctional EB, and dystrophic EB and is associated with cutaneous deformities and mucosal site strictures.  Skin cancer, a known complication, is widely reported in the literature.  Inherently poor wound healing complicates treatment of these carcinomas.  Though esophageal strictures and growth retardation are commonly seen,  association of epidermolysis bullosa with squamous cell carcinoma of the esophagus is rare.
An 81-year-old Indian male, a known case of epidermolysis bullosa with active cutaneous lesions [Figure 1], presented with a three-month history of progressively increasing dysphagia. On endoscopy, a proliferative mass was seen 20 cm from incisor teeth, with complete obstruction of esophageal lumen. A biopsy from the lesion revealed squamous cell carcinoma, grade 2. There was no radiological evidence of mediastinal involvement in contiguity with the tumor and no discernable thoraco-abdominal lymphadenopathy. Therefore, a diagnosis of squamous cell carcinoma esophagus T3 N0 M0 was made. External beam radiotherapy was planned after endoscopy-guided Ryles' tube insertion for maintenance of enteral nutrition. Concurrent chemotherapy was not considered due to co-existent EB portending an increased risk of unacceptable muco-cutaneous toxicity.
Details of Radiotherapy
Volumes: [Figure 2]
GTV included radiologically demonstrable tumor on CT scan
CTV1 included the GTV with 10 mm radial margins and 40 mm supero-inferior margins along the esophagus
CTV boost included the GTV with 10 mm radial margins and 25 mm supero-inferior margins
PTV1 and PTV boost included the respective CTVs with 10 mm margin [Figure 2]
The paratracheal and supraclavicular nodal stations were included in the nodal CTV
A 10 mm margin was grown to realize the PTV node.
Dose: PTV1: 39.6Gy in 22 fractions, PTV Node: 54Gy in 30 fractions, PTV boost : 61.2Gy in 34 fractions
Multiple fields were used to plan the case, respecting cord, lung and cardiac dose constraints and with special consideration towards reducing the cutaneous dose. The patient tolerated treatment fairly well with development of Grade 2 skin toxicity towards the fifth week of radiotherapy. Dysphagia gradually improved from second week of treatment. However, the patient again started complaining of worsening dysphagia during the sixth week of radiotherapy. The patient was managed conservatively with a course of antibiotics and steroids following which the dysphagia was relieved. The toxicity was comparable to other non-EB patients treated with radiotherapy where a Grade 2 skin toxicity and moderate dysphagia is commonly observed while on treatment. The patient is currently undergoing follow up. He had no dysphagia when last examined six weeks after completion of treatment. Endoscopic and radiological evaluation has been advised for objective assessment of response.
This case is reported for several unique features, which include the rare occurrence of carcinoma esophagus with epidermolysis bullosa, and acceptable treatment induced muco-cutaneous toxicity.
The treatment of carcinoma of cervical esophagus in EB is complicated by the increased risk of iatrogenic muco-cutaneous toxicity. However, we report the successful completion of treatment in one such case with external beam radiotherapy alone with comparable muco-cutaneous toxicity and significant subjective improvement of dysphagia. Special care to spare skin and uninvolved mucosa is warranted while planning such cases.
| » References|| |
|1.||Uitto J, Richard G. Progress in epidermolysis bullosa: From eponyms to molecular genetic classification. Clin Dermatol 2005;23:33-40. [PUBMED] |
|2.||Yamada T, Suzuki M, Hiraga M, Toda S, Fujimoto M, Ohtsuki M, et al . Squamous cell carcinoma arising on scars of epidermolysis bullosa acquisita. Br J Dermatol 2005;152:588-90. [PUBMED] [FULLTEXT]|
|3.||Bastin KT, Steeves RA, Richards MJ. Radiation therapy for squamous cell carcinoma in dystrophic epidermolysis bullosa: Case reports and literature review. Am J Clin Oncol 1997;20:55-8. [PUBMED] [FULLTEXT]|
|4.||Fine JD, Johnson LB, Weiner M, Suchindran C. Gastrointestinal complications of inherited epidermolysis bullosa: Cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastroenterol Nutr 2008;46:147-58. [PUBMED] [FULLTEXT]|
[Figure 1], [Figure 2]
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