Letters to Editor
interval than patients in whom resection was incomplete with 83 versus 60% five-year relapse-free interval. Adjuvant radiotherapy seems to have only little effect on recurrence and chemotherapy has been ineffective up to now.[2,5] Spear et al found local control rates of 77% with radical surgery alone versus 100% for patients with surgery and radiation after microscopically complete resection.[6] So, the overall prognosis depends mainly on the localization of the process. Neurosurgeons are seldom confronted with extraperitoneal aggressive fibromatosis of the desmoid type. It is necessary to know the essentials of this neoplasm in order to manage it correctly. Complete surgical removal still remains the treatment of choice; whereas radiation therapy is reserved for situations in which gross resection is not possible because of cosmetic or functional reasons.
Th is a PD si F te i ho s a st va (w ed ila w by ble w .m M fo ed ed r fr kn kn ee ow ow do .c Pu w om b nl o ). lica ad tio fr ns om
microscopic picture. There was a tendency to infiltrate the peritumoral muscle, which showed degeneratively changed giant cells. The diagnosis of a nuchal extraabdominal aggressive fibromatosis of desmoid type was established. Aggressive fibromatosis is a rare tumor of the soft tissue with an incidence of two to four per million. Normally neurosurgeons are seldom confronted with this entity.[1] In general it is a tumor of younger patients. Mean age at presentation is 30 to 45 years.[2,3] Our 77-year-old patient is extraordinarily old. Because of its destructive growth into the surroundings the tumor is semimalignant.[2,4] Distant spread hasn’t been observed yet, but significant mortality can result from local tumor complications. Total tumor resection within healthy tissue is therefore the most important prognostic factor, otherwise the rate of local recurrences is high.[1,2] Clinically, patients suffer from an indolent mass relatively fast growing sometimes causing symptoms by compressing adjacent structures, e.g. the plexus brachialis.[1] The shoulder, upper arm and the gluteal region is the main location in children. Our patient presented the typical history, but the location in the neck was unusual. Mostly females are affected with a percentage of about 66% of all described cases.[2] Magnetic resonance imaging is the method of choice to visualize the tumor with its borders. Conventional X-ray of the region often shows a diffuse soft tissue swelling. Possible differential diagnoses are inflammatory changes such as fasciitis nodularis and other lesions such as fibromas, leiomyosarcomas, myopericytomas, myofibroblastomas, chondroblastomas, metastatic spindle cell carcinoma of the breast and myomas.[2] But the predominant fibrous differentiation or a differentiation along smooth muscle was not observed in our patient’s tumor material. Some describe aggressive fibromatosis to be a lesion that imposes with homogenous isointensity or mild hyperintensity in T1-weighted sequences of MRI.[3] In our case there was a mild hyperintensity on T1-sequences [Figure 1A]. A heterogeneous high signal was seen in T2-sequences [Figure 1C]. All the lesions published enhanced inhomogenously gadolinium after intravenous administration.[3] The homogenous enhancement of our patient’s tumor could be related to a high dosage of gadolinium or the very high cellularity of the lesion. Another prominent difference was the appearance of the tumor as a discrete mass in comparison to an infiltrative ill-defined enlargement of a muscle group in other cases.[3] Gross total resection within healthy tissue is the treatment of choice. It is the only well-known prognosis parameter. [2,3,5] Incomplete resection seems to be associated with a higher rate of recurrence. The overall recurrence rate is 35-60%. Patients with complete tumor resection had a significantly longer recurrence-free 420
Christian Ewald, Susanne A. Kuhn, Michael Brodhun*, Rolf Kalff
Departments of Neurosurgery and *Institute of Pathology, Medical Center of Friedrich-Schiller-University, Jena, Germany. E-mail:
[email protected]
References
1. Seinfeld J, Kleinschmidt-Demasters BK, Tayal S, Lillehei KO. Desmoidtype fibromatoses involving the brachial plexus: Treatment options and assessment of c - KIT mutational status. J Neurosurg 2006;104:749-56. 2. Kuhnen C, Helwing M, Rabstein S, Homann HH, Mueller KM. Desmoidtype fibromatosis (aggressive fibromatosis). Pathologe 2005;26:11726. 3. Lee JC, Thomas JM, Philips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol 2006;186:247-54. 4. Ferenc T, Sygut J, Kopczynski J, Mayer M, Latos-Bielenska A, Dziki A, et al. Aggressive fibromatosis (desmoid tumours): Definition, occurrence, pathology, diagnostic problems, clinical behaviour, genetic background. Pol J Pathol 2006;57:5-15. 5. Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD. Desmoid tumours and current status of management. Orthop Clin North Am 2006;37:53-63. 6. Spear MA, Jennings LC, Mankin HJ, Spiro IJ, Springfield DS, Gebhardt MC, et al. Individualizing management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys 1998;40:637-45. Accepted on 23-05-2007
Paradoxical progression of conus tuberculoma during chemotherapy of tuberculous meningitis Sir, Simultaneous presentation of both spinal arachnoiditis and intramedullary tuberculoma, which deteriorate Neurology India | October-December 2007 | Vol 55 | Issue 4
Letters to Editor
Th
is a PD si F te i ho s a st va (w ed ila w by ble w .m M fo ed ed r fr kn kn ee ow ow do .c Pu w om b nl o ). lica ad tio fr ns om
during antituberculous chemotherapy, is too rare. We report a rare case and study the clinico-pathological manifestations of spinal arachnoiditis and intramedullary tuberculoma. A 21-year-old girl was admitted to our hospital due to fever and increased headache since one-and-ahalf month before admission. The patient appeared ill and had neck stiffness. Chest X-ray was normal. A plain brain CT scan revealed dilated ventricles with periventricular edema. Cerebrospinal fluid analysis yielded 2600 leukocytes/µl of which 70% were lymphocytes; total protein level was 240mg/dL, glucose level 40mg/dL. Simultaneous blood glucose was 110mg/dL. No organisms were seen on gram stain or in AFB (acid-fast bacilli) smear of CSF and gastric lavage fluid. Polymerase chain reaction (PCR) test specific for M. tuberculosis was reported positive. HIV antibody was negative. The patient was started on four-drug antituberculous chemotherapy and steroid. Neurological consultation revealed progressive loss of consciousness, symmetric movements of the limbs, increased deep tendon reflexes, neck stiffness, Babinski sign and blurred optic discs. A medium-pressure ventriculo-peritoneal shunt was inserted immediately. Over four weeks postoperatively she got better but she couldn’t move her feet perfectly and got blind. Left abductor palsy was noted. Motor function was graded 2/5+ proximally and 1/5 distally in both lower limbs with bilateral Babinski sign. Deep tendon reflexes were absent. Another CSF analysis yielded the same result. Electromyographic study revealed secondary axonal injury of the tibialis and common peroneal nerves due to multiple lumbosacral radiculopathies. On myelography a huge filling defect was present at the level of L1-L2 vertebrae, candle guttering appearance at the level of T11-12 and root adhesion of the cauda equina region [Figure 1]. The surgical procedure included laminectomies at T11-L3, dural incision and midline posterior myelotomy at the level L1-2. There was white to yellow cheesy soft suctionable tissue including small calcified particles. Pathologic tissue extended from the conus medullaris to cauda equina. Histopathologic examination revealed granulomatous lesion that contained caseating necrosis, Langhans’ giant cells and perivascular lymphocytic infiltration in the specimens. The first documented case of paradoxical reaction during treatment of tuberculosis was reported by Citow et al.[1] and similar cases have been reported occasionally.[2,3] These phenomena generally occurred within three months after treatment initiation. Decreased penetration of antituberculous drugs into the brain or spinal cord and restoration of blood-brain
Neurology India | October-December 2007 | Vol 55 | Issue 4
Figure 1: Anteroposterior and lateral view myelogram shows candle guttering (white arrow) and tuberculomas (black arrows) in the thoracolumbar region
barrier might lead to reactivation of latent foci. It does not usually represent treatment failure but the most likely explanation for these phenomena is an interaction between the host’s immune response and the direct effects of mycobacterial products. This suggest that despite an adequate response to treatment, an ongoing inflammatory process takes place in the arachnoid and spinal cord tissue. [4] Campbell proposed that rapid killing of bacilli by effective treatment can cause the release of large amount of tuberculoprotein and other cell-wall products.[5] It is logical to assume that the overall inflammatory response to M. tuberculosis reflects both the number and function of the appropriate immune cells and the amount of the antigen that they encounter.[2]
Ali Moghtaderi, Roya Alavi-Naini*, Vafa Rahimi-Movaghar**
Departments of Neurology, Zahedan, and *Infectious Diseases, Zahedan and **Neurosurgery, Tehran University School of Medical Sciences, Zahedan, Iran. E-mail:
[email protected]
References 1. Citow JS, Ammirati M. Intramedullary tuberculoma of the spinal cord: Case report. Neurosurgery 1994;35:327-30. 2. Breen RA, Smith CJ, Bettinson H, Dart S, Bannister B, Johnson MA, et al. Paradoxical reactions during tuberculosis treatment in patients with and without HIV co-infection. Thorax 2004;59:704-7. 3. Devi BI, Chandra S, Mongia S, Chandramouli BA, Sastry KV, Shankar SK. Spinal intramedullary tuberculoma and abscess: A rare cause of paraparesis. Neurol India 2002;50:494-6. 4. Skendros P, Kamaria F, Kontopoulos V, Tsitouridis I, Sidiropoulos L. Intradural, extramedullary tuberculoma of the spinal cord as a complication of tuberculous meningitis. Infection 2003;31:115-7. 5. Campbell IA, Dyson AJ. Lymph node tuberculosis: A comparison of various methods of treatment. Tubercle 1977;58:171-9. Accepted on 18-05-2007
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