Case Study 37 Henry Armah, M.D., M.Phil.
Question 1 Clinical history: 75-year-old female with history of back pain and bilateral lower extremity weakness. He was a non-smoker, occasionally used alcohol socially, and did not use illicit drugs. Spinal MRI revealed a large (6.0 cm in largest dimension) extra-axial intradural mass at the level of T10-11 vertebrae. The mass was completely resected at an outside hospital and the permanent section sent to you for consultation. Describe the microscopic findings on this H&E slide? Click here to view slide.
Answer Fragments of a malignant pleomorphic tumor with both spindle and epithelioid components. The epithelioid component appears to focally form gland-like structures. Mitotic figures are readily identified.
Question 2 What are your differential diagnoses based on the patients’ age and histological findings?
Answer Malignant Peripheral Nerve Sheath Tumor High-grade Glioma
Question 3 What additional studies would you need to rule out other important differential diagnoses and confirm the final diagnosis in this case?
Answer GFAP, S-100, Neurofilament, and Ki-67 (MIB-1).
Question 4 What do you see on this S-100 immunostain slide? Click here to view slide.
Answer S-100 is moderately positive in scattered spindled and epithelioid tumor cells.
Question 5 What do you see on this GFAP immunostain slide? Click here to view slide.
Answer GFAP is negative in tumor cells.
Question 6 What do you see on this Neurofilament immunostain slide? Click here to view slide.
Answer Neurofilament is weakly positive in rare tumor cells.
Question 7 What do you see on this Ki-67 (MIB-1) immunostain slide? Click here to view slide.
Answer Ki-67 (MIB-1) is positive in the nuclei of up to 25% of tumor cells.
Question 8 What is your final diagnosis in this case?
Answer Malignant Peripheral Nerve Sheath Tumor
Question 9 Which of the following familial tumor syndrome is this lesion commonly associated with? Turcot syndrome Neurofibromatosis type 1 Neurofibromatosis type 2 Von Hippel-Lindau disease Li-Fraumeni syndrome
Answer B. Neurofibromatosis type 1