Fibrosarcomatous Dermatofibrosarcoma Protuberans of the Head.

This article highlights the case of a 37-year-old male who presented with a recurrent, exponentially enlarging head mass, emphasizing the diagnostic and therapeutic challenges associated with a very rare type of tumor, fibrosarcomatous dermatofibrosarcoma protuberans (DFSP) of the head. Our patient presented with a rapidly growing head mass, initially diagnosed as a spindle cell tumor, and was managed with surgical excision and skin flap grafting. Follow-up revealed relapse and interval development of hemiparesis and hemisensory loss. MRI revealed tumor recurrence, with compression of the right parietal lobe and superior sagittal sinus. Histopathology revealed stroma with fascicles of spindle cells homogenous to fibrillar cytoplasm, with oval vesicular nuclei. Immunohistochemical staining showed positivity for CD34 and SMA. Oral chemotherapy with imatinib 800 mg/day was started. Follow-up imaging showed a marked reduction in the size of the tumor and resolution of the compression of the underlying brain parenchyma with cystic degeneration and decreased contrast enhancement. Future plans include possible surgical tumor debulking and/or radiation therapy. Although extremely rare, awareness of this tumor, with a multi-disciplinary approach to the management of the case, is vital to maximize treatment outcomes.

Understanding the Brain-Heart Connection Through a Case of Angry Glioma Syndrome.

We discuss a patient with a tumor on the anterior corpus callosum who underwent open biopsy eventually succumbing to cerebrogenic fatal arrhythmia following wounded glioma syndrome. A healthy 37-year-old female patient was admitted to our department due to a history of headache for 13 months. MRI revealed a suspicious glioma infiltrating the anterior corpus callosum. Neurologic examination only showed low cognitive assessment score (Montreal Cognitive Assessment score 20/30). ECG was normal sinus rhythm. Steroids and levetiracetam were administered prior to operation. Patient underwent right frontal craniotomy and biopsy of tumor with unremarkable events. During the first hospital day, patient had episodes of bradycardia followed by decrease in sensorium. Brain CT scan showed progression of edema without hemorrhage within the tumor bed. This was followed minutes later by two episodes of generalized tonic-clonic seizures and pulseless ventricular tachycardia. Cardiac resuscitation was done for 24 minutes but patient eventually expired. Location of the lesion and the epileptogenicity of the peritumoral cortex greatly contributed to the patient's demise. Involvement of the fronto-mesial structures, particularly the insula and the cingulate cortex, and their connection to the central autonomic network, increased susceptibility to arrhythmias. Decreased seizure threshold worsened post-operative edema, further aggravating the dysregulation of the brain-heart-connection.

Giant Cell Tumor of the Clivus in a 19-Year-Old Male Presenting With Doubling of Vision: A Case Report.

Giant cell tumor (GCT) of the skull is an extremely rare condition, accounting for less than one percent of all bone GCTs. Clival GCT is even rarer, with only 25 cases documented to date. It generally follows a benign course; however, due to its location and vascularity, it can be locally aggressive. Complete resection of GCT in this location may be challenging, resulting in residual tumors. In this paper, we report a case of a 19-year-old male who presented with a chronic headache later accompanied by diplopia and was noted to have a mass spanning the sella and the clivus on cranial imaging. The histopathology report of the excised mass revealed findings compatible with GCT of the bone. Most GCTs remain stable in the first two years after initial treatment. However, four months after its partial excision, the clival GCT continued to progress. The patient underwent adjuvant radiation therapy, yet symptoms persisted. This profile highlights the crucial role of long-term surveillance and prompt adjuvant radiation therapy and chemotherapy.

Concurrent Wounded Glioma Syndrome and Distant Wounded Glioma Syndrome Following a Gross Total Resection of Glioblastoma: A Case Report.

Surgery is the initial form of treatment for glioblastoma, and a maximum resection without impairing neurological function improves survival. Wounded glioma syndrome (WGS) is a clinical picture observed after the resection of high-grade tumors. This syndrome, developing within hours to a few days after glioma surgery, is characterized by hemorrhage into the postoperative cavity and cerebral edema and at times occurs in areas distant from the site of the resection, i.e., distant wounded glioma syndrome (DWGS). We report a case of a 70-year-old male presenting with acute-onset left leg weakness, with a large peripherally enhancing mass with central non-enhancement suggestive of necrosis in the right frontal lobe. A gross total resection of the tumor was done, and the histopathologic evaluation verified the diagnosis of glioblastoma World Health Organization (WHO) grade IV. During the postoperative period, he was drowsy and was able to move his right extremities. He had a series of generalized tonic-clonic seizures three hours after the operation. After eight hours, the patient became comatose with signs of increasing intracranial pressure. A cranial computed tomography (CT) scan revealed diffuse cerebral edema and hemorrhage into the operative site in the right frontal lobe, as well as subarachnoid hemorrhages in the bilateral frontoparietal sulci. There were also small hemorrhages seen in the left caudate head, midbrain, and left hemipons. Death occurred the following day. This case report demonstrates an unusual case of a WGS with a concurrent DWGS in the brainstem after a gross total resection of a frontal lobe glioma. This case shows a significantly uncommon sequela that a patient undergoing glioma surgery can present, leading to rapid deterioration and death. Resection of a glioma carries a significant risk, and its impact in the immediate postoperative period merits evaluation when planning perioperative management, taking prompt action if these syndromes occur.

Differentiating Cerebral Toxoplasmosis and Tumor Recurrence by Thallium-201 Single-Photon Emission Computerized Tomography in a 28-Year-Old Female with Astrocytoma.

Cerebral toxoplasmosis is an opportunistic infection that, by itself, is difficult to differentiate from cerebral neoplasms by conventional neuroimaging. It rarely occurs concurrently in patients with a primary brain tumor but when it does, it makes diagnosis and management more difficult. This is a case of a 28-year-old female, diagnosed with a right frontal pleomorphic xanthoastrocytoma with several recurrences, treated with surgery, radiation, and chemotherapy. Three years from diagnosis, the patient was readmitted for generalized body weakness, fever, and a decrease in sensorium. A repeat cranial magnetic resonance imaging showed multiple enhancing lesions in both cerebral hemispheres and in the posterior fossa. Serum toxoplasma IgM and IgG antibody titers were elevated. Single-photon emission computerized tomography (SPECT) with thallium-201 did not show increased tracer uptake in these lesions, favoring toxoplasmosis over tumor recurrence. The patient was treated with trimethoprim-sulfamethoxazole with significant improvement. This is a rare account of cerebral toxoplasmosis arising in the setting of astrocytoma. This is also the first case report to demonstrate the value of thallium-201 SPECT in differentiating central nervous system infection from tumor recurrence, which is pivotal in management. More studies exploring the use of thallium-201 SPECT in distinguishing central nervous system infections from glioma and other malignant tumors should be undertaken to maximize this imaging modality in neuro-oncology practice.