An Unusual Presentation of Abrikossoffs Tumor: A Case Report

Introduction

Granular cell tumors is a rare benign tumor described for the first time by Abrikossoff in 1926 in a patient with a tongue tumor [1]. Today it is considered that this tumor is probably derived from Schwann cells [2]. It occur mostly in adults between fourth and sixth decade, rarely in children [3].  The most common sites are the head and neck regions (45-65%) [4], it also can be found in skin, gastrointestinal tract, respiratory tract, nervous system, male and female reproductive system [5].

The malignant GCT (mGCT) form is an extremely rare entity, representing approximately 2% of all GCT, and it presentation on the spine is exceptionally rare localization.

We present a case of GCT located on the sacral canal with intramedullary extension.

Case Report

A 42 yeas old women was referred to a neurology department after presenting a back pain evolving for more than two years and lately worsened with movement of legs. The preoperative lumbar magnetic resonance imaging (MRI) show a intramedullary sacral tumor with a double component which was isointense on T2-weighted images, hyperintense on T1-weighted images, and infiltrating the sacral bone structures, the tumor was suggestive of an ependymoma. 

The patient then had decreased superficial sensations along with paraplegia in both the lower limbs. A lumbar CT-Scan was performed showing cauda equina compression located on S2. A biopsy was then performed and the final diagnostic was made on the surgical resection, the Immunohistochemical (IHC) stains were positive for CD68, S100, CD163 and SOX10. There was no reaction with epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP) and the Ki67<10% The pathologist concluded, after a review with an expert center in USA to a malign Abrikossoff tumor.

Complete excision of the tumour could not be achieved owing to its location. The patient was then treated by adjuvant radiotherapy on the residual tumor of 50 Gy in conventional fractionation with the aim of shrinking the tumor and obtaining a local control. 

Figure 1: Sacral tumor after resection.

Figure 2: Planning review for 3D radiotherapy: computed tomography scan showing the target volumes: GTV in red, PTV-HR in yellow. (C) delivered dose to the region of high risk, was 50Gy Gy (95% isodose line in red). GTV, gross tumor volume, PTV, planning target volume; HR, high risk.

The follow-up MRI showed a local-regional control. The patient is asymptomatic and her paraplegia was resolved.

Discussion

GCT, also called granular cell myoblastomas is first described by the Russian pathologist Abrikossoff in 1926, involving multiple anatomical sites, but only few cases has been described in spinal cord [6-11].

The histogenesis of Granular cell tumor is unknown. Tumors are believed to be caused by alterations in Schwann cell metabolism. This hypothesis is supported by the persistent presence of the S-100 protein on immunohistochemistry which is also the case for our patient. Clinical symptoms are linked to the tumor site. For tumor who are located in the nervous system, the symptomatology appears as radicular pain, or burning pain, loss of sphincter tone (with bowel and bladder dysfunction), paresthesia etc [7-11].

This spinal tumor are best evaluated by MRI. Tumors are typically slightly hypointense on T1-weighted sequences and show uniform contrast enhancement after intravenous injection of gadolinium. They are generally hypointense on T2-weighted sequences. But similar changes are also seen in other spinal tumors, therefore The histopathologist is responsible for the final diagnosis.

Histopathologically, GCTs consist of large round or polygonal cells with small, dense nuclei. The cytoplasm is enriched with abundant eosinophilic granular substance witch are periodic acid-Schiff (PAS)-positive. Other cancer may also have granular cytoplasm but have other histologic and immunohistochemical features that distinguish them from Abrikossoff tumors [12].

Immunohistochemical analysis provided diagnostic confirmation of granular cell tumour by showing an over expression of S-100 protein, CD68, NSE, inhibin-α, and vimentin. The cells fail to react with GFAP, neurofilament protein (NFP), HMB-45, keratin, EMA, CK7, chromogranin, and synaptophysin. CGTs are usually benign neoplasm, but only few cases 1-10% are malignant. Characteristics that stipulate malignant GCT are, tumor size, rapid growth, presence of metastasis, necrosis, and involvement of adjacent tissue.  

Surgical excision is the treatment of choice for malignant CGT. The surgery should accomplished a healthy margins with a 2-3 cm safe margins. In case of incomplete excision, the patient should undergo a revision [13-15], but in some cases, due to the localization, this couldn’t be achieved, and an adjuvant treatment should be considered.

Radiotherapy and chemotherapy have historically been used when malignant tumors are present or when tumors with wide margins cannot be completely resected, but their efficacy has not yet been proven. Although rarely reported, in one case radiotherapy was successfully used to stabilize recurrent/residual disease [17].

This is the case for our patient who a complete resection could not be achieved, and an adjuvant radiotherapy was done (Fgure 1 and 2).

Conflict of Interest Statement

The authors declare no conflict of interest.

References

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