Malignant Transformation of Neurofibromatosis
in A Developing Community: Case Report
Wilson IB Onuigbo*
Department of Pathology, Medical Foundation & Clinic, Nigeria
Submission: July 04, 2018; Published: July 26, 2018
*Corresponding Address: Wilson IB Onuigbo, Department of Pathology, Medical Foundation & Clinic, 8 Nsukka Lane, Enugu 400001, Nigeria, Email: firstname.lastname@example.org
How to cite this article: Wilson IB O. Malignant Transformation of Neurofibromatosis in A Developing Community: Case Report. Canc Therapy & Oncol
Int J. 2018; 11(4): 555816. DOI: 10.19080/CTOIJ.2018.11.555816
It has been mooted that the establishment of a histopathology data pool facilitates epidemiological analysis. Therefore, such a pool, which was established in a developing community, was used to study biopsy cases of neurofibromatosis with special reference to those in which transformation of malignancy was suspected. Two such examples were found among some 163 random cases. They deserve documentation.
Keywords: Histopathology; Data pool; Analysis; Neurofibromatosis; Transformation; Malignancy; Igbos; Nigeria
A Birmingham (UK) group hypothesized that the establishment of a histopathology data pool cannot but facilitate epidemiological analysis . Therefore, since such a data pool was established at Enugu, the erstwhile capital city of the Eastern Region of Nigeria, can it be used to answer a genetic question? What of the Igbo ethic group ? This was answered personally as the pioneer pathologist who had encouraged local practitioners to send specimens provided the epidemiologic data were furnished in special Histology Request Forms.
EO, a 44-year-old woman, attended the Out-Patient Clinic run by Dr J.O. Ojukwu at the University of Nigeria Teaching Hospital, Enugu. The largest of her masses measured 5 cm by 5 cm and was situated on the right anterior chest wall. It was of 10 years duration. With the provisional diagnosis as “Neurofibromatosis with malignant transformation,” excision biopsy was carried out. A 4.5 cm bosselated skin mass with underlying soft yellowish tumor tissue was received personally. On microscopy, the background was of benign tumor with neuroid elements. There were mitotically active, spindle cells manifesting hyperchromatism.
MJ, a 25-year-old man, attended the same Hospital under Dr D.C Nwafo with the complaints of swellings in the back of the chest and right thigh as well as all over the body. He was provisionally diagnosed as “Neurofibromatosis with malignant transformation.” A 13 cm dome shaped sessile tumor was museum worthy on submission. There were other submitted irregular pieces including skin. On microscopy, some parts were
benign with wavy spindle nuclei as well as mitotically active elements showing pleomorphism and hyperchromatism.
Both manifestations were confirmed to have undergone malignant change. In this context, Internet searches yielded neurofibromas which became malignant in USA , and UK . There were also UK & Canada joint examples . According to French authors , “The majority are indolent, but factors predictive of poor prognosis are yet to be identified.” Hopefully, USA authors  recommended that “Multidisciplinary care at a high-volume sarcoma center is essential for optimal outcomes.” “Further work,” they added, “is needed to develop targeted combinational therapies for these tumors.”