Abrikossoff's tumour or granular cell tumour is a very rare entity that was first described in 1926 by the russian pathologist Abrikossoff. Benignity is the rule with only a few cases of malignancy reported. It is frequently presented as a single ulcerated well-defined nodule with progressive growth. Its etiology is unknown but nowadays the consensus is that Abrikossoff's tumour has a neural or nerve sheath origin.
Most common locations are the head and neck (45% - 65%), from which more than half are located in the oral cavity. Although other locations can also be affected, Abrikossoff's tumour presented on the upper limbs is the rarer.
In this setting, me and fellow surgeons were pleased to have our work about an Abrikossoff's tumour on the upper limb published in BMJ Case Reports.
In 2017, Costa Almeida et al. were asked to treat a male patient with a pinkish well-defined non-ulcerated nodule on the left upper limb, painless, with progressive growth up to 3 cm. A core biopsy diagnosed an Abrikossoff's tumour. The authors decision was to proceed to surgical resection using a Limberg flap procedure with local anaesthesia. Cicatrisation was perfect. Pathology confirmed the diagnosis of a benign Abrikossoff's tumour. In a PubMed research the authors only found 21 case reports of Abrikossoff's tumour on the upper limbs.
Because this rare tumour has non-specific symptoms, histological findings are essential for diagnosis. Abrikossoff's tumour can be confused with squamous cell tumour, and when epidermis is invaded differentiation with melanoma may not be easy. It can also be misdiagnosed with leiomyoma, dermatofibrosarcoma or angiosarcoma if only light microscopy is used. In this setting, immunohistochemistry is very important to increase pathology accuracy.
Standard treatment is surgical resection with free margins, and recurrence is unusual in benign cases. However all surgeons must be aware that malignancy is a possibility and that only the presence of metastasis is an accepted criterion for malignancy. Additionally, malignant Abrikossoff's tumours tend to metastasise and relapse 1 year after surgical treatment, which emphasises the importance of a long-term follow-up. Doctors must not forget to examine the whole patient since Abrikossoff's tumour can arise in many locations throughout the human body.
Rare things do exist, so learn about them...
Link to PubMed:
Dr. Carlos Eduardo Costa Almeida
General Surgeon