Major intratumoral hemorrhage of a petroclival atypical meningioma: case report and review of literature.
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ABSTRACT:
SUBMITTER: Mangubat EZ
PROVIDER: S-EPMC3134810 | biostudies-other | 2010 Nov
REPOSITORIES: biostudies-other
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Project description:BackgroundIntramedullary hemorrhages involving spinal hemangioblastomas are rare. They are frequently associated with devastating neurologic outcomes, despite with emergent surgical intervention. Here, we presented an example of an intramedullary hemorrhage occurring in a spinal hemangioblastoma, where the patient markedly improved with surgery. Additionally, the appropriate literature was reviewed (including intraoperative video).Case descriptionA 49-year-old female with a 4-year history of tingling in the left lower extremity presented with vomiting, stepwise worsening of bilateral scapular pain, new upper motor neuron signs, and severe sensory loss bilaterally below C4 on the left and T4 on the right. The magnetic resonance imaging demonstrated a well-circumscribed, uniformly enhancing intramedullary tumor at the C2 level with hyperintensity on the T2 study consistent with acute hemorrhage and cord edema. An urgent C2 laminectomy was performed for gross total tumor resection. Intraoperatively, intramedullary hemorrhage was identified anterior to the tumor mass and was confirmed histopathologically. Postoperatively, the patient had no new sensorimotor deficits and fully recovered within two postoperative months.ConclusionsPatients presenting with acute intramedullary hemorrhage within hemangioblastomas of the spinal cord may demonstrate significant postoperative neurological recovery.
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Project description:Cystic meningiomas are very rare tumors of the central nervous system. We report the case of a 62-year-old female how presented headaches resistant to usual analgesics with behavior disorders. Cerebral CT scan showed a right frontal extra-axial tumor with firm and cystic component, brain MRI evoked the diagnosis, surgery removed the entire tumor and histological examination confirmed it. A cystic meningioma should not be omitted from the differential diagnosis of brain tumors with a cystic component and which clinical, radiological, histological and therapeutic features are discussed.
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Project description:Bullous pemphigoid (BP) is a rare, life-threatening autoimmune blistering disease with pruritus and tension blisters/bullous as the main clinical manifestations. Glucocorticosteroids are the main therapeutic agents for it, but their efficacy is poor in some patients. Tofacitinib, a small molecule agent that inhibits JAK1/3, has shown incredible efficacy in a wide range of autoimmune diseases and maybe a new valuable treatment option for refractory BP. To report a case of refractory BP successfully treated with tofacitinib, then explore the underlying mechanism behind the treatment, and finally review similarities to other cases reported in the literature. Case report and literature review of published cases of successful BP treatment with JAK inhibitors. The case report describes a 73-year-old male with refractory BP that was successfully managed with the combination therapy of tofacitinib and low-dose glucocorticoids for 28 weeks. Immunohistochemistry and RNA sequencing were performed to analyze the underlying mechanism of tofacitinib therapy. A systematic literature search was conducted to identify other cases of treatment with JAK inhibitors. Throughout the 28-week treatment period, the patient experienced clinical, autoantibody and histologic resolution. Immunohistochemical analysis showed tofacitinib significantly decreased the pSTAT3 and pSTAT6 levels in the skin lesions of this patient. RNA sequencing and immunohistochemical testing of lesion samples from other BP patients identified activation of the JAK-STAT signaling pathway. Literature review revealed 17 previously reported cases of BP treated with four kinds of JAK inhibitors successfully, including tofacitinib (10), baricitinib (1), upadacitinib (3) and abrocitinib (3). Our findings support the potential of tofacitinib as a safe and effective treatment option for BP. Larger studies are underway to better understand this efficacy and safety.
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Project description:BackgroundPrimary intraosseous meningioma is a subset of extradural meningioma that arises in the bone, and only a few cases have been reported to date.Case presentationAn 80-year-old man presented with decreased hearing on the right side accompanied by a disturbance of balance 10 months prior to admission. Magnetic resonance imaging revealed an 8 × 7 cm osteolytic mass in the right posterior fossa related to the petrous bone, with extension to the cervical region. During surgery, the tumor was found to be located extradurally, with no invasion of the dura. The tumor was removed entirely, apart from a small portion around the jugular foramen to avoid lower cranial nerve injury.ConclusionThe final diagnosis was primary intraosseous osteolytic meningioma with atypical pathology. Here, we report a rare case of an osteolytic skull lesion in the skull base not invading the dura and with extensive bone destruction.
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Project description:Background Chordomas are rare, locally aggressive neoplasms thought to arise from notochordal remnants in the axial skeleton. Primary intradural chordomas are considered to be extremely rare. In this article a giant intradural petroclival chordoma is presented, and a synthesis of the available literature is performed to measure overall survival (OS) and recurrence-free survival (RFS) and to identify prognostic factors. Methods A systematic Medline review yielded 47 patients with purely intradural tumors from 38 publications including 39 chordomas, 8 cases of ecchordosis physaliphora, and 1 case with features of both. The 5-year OS and RFS were calculated based on the Kaplan-Meier method. Risk factors for progression or mortality were analyzed using binomial logistic regression. Results Maximal tumor diameter varied from 1.5 to 6.0 cm (mean: 3.2 cm). Tumors were located predominantly in the prepontine area (66.7%). Combined 5-year Kaplan-Meier OS and RFS were 77% ± 11% and 74% ± 11%, respectively. Incomplete surgical resection, larger tumor diameter, and an elevated Ki-67 index were statistically more frequent in cases of recurrence and mortality. Conclusions Based on a systematic literature review, the behavior of primary intradural chordomas may be closer to typical chordomas than was previously thought.
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Project description:Introduction and importanceMeningiomas are extra-axial central nervous system (CNS) tumors that arise from the arachnoid cells of the dura mater. Only 1.8-3.2% of all meningiomas are located at foramen magnum (FM) and pure posterior FM meningioma are very rare. The diagnosis of malignancy in patients with meningiomas has been a controversial issue. Only a histological study can confirm this situation.Case presentationWe report a case of A 52-year-old female presented with a history of neck pain with progressive spastic quadriparesis.Clinical discussionMagnetic resonance imaging MRI T1 and T2 weighted images revealed well-defied pure posterior foramen magnum Lesion. Although the lesion was very sticky to neurovascular components. Simpson grade I was achieved. Histopathology revealed Chordoid meningioma. The patient had a dramatic recovery.ConclusionAlthough choroid meningioma is usually well circumscribed, sticky tumors should be suspected. Recurrence of Chordoid meningioma should be suspected. Total excision should be achieved and routine follow-up should be informed. Reports about chordoid meningioma aren't common, but reports on choroid foramen magnum meningioma are very rare. The opportunity to give the patient a symptom-free and normal life should not be missed in such cases.
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Project description:BackgroundGout is a common metabolic disorder of purine metabolism, causing arthritis in the distal joints of the appendicular skeleton. Spine involvement is rare, and very few cases of spinal gout have been reported. The authors present a rare case of axial gout with tophaceous deposits in the thoracic spinal canal resulting in cord compression and mimicking a meningioma.Case descriptionA 33-year-old male presented with chronic mid back pain and a progressive paraparesis. The presumed diagnosis was meningioma based on MR imaging with/without contrast that showed a posterolateral, right-sided, and T10-T11 intradural extramedullary lesion. Notable, was hyperuricemia found on hematological studies. The patient underwent a decompressive laminectomy (T9-T11) for excision of the lesion, intraoperatively, an intraspinal, chalky, white mass firmly adherent to and compressing the dural sac was removed. The histopathology confirmed the diagnosis of a gouty tophus. Postoperatively, the patient's pain resolved, and he regained the ability to walk.ConclusionA gouty tophus should be included among the differential diagnostic considerations when patients with known hyperuricemia present with back pain, and paraparesis attributed to an MR documented compressive spinal lesion.
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Project description:Introduction and importanceEn-plaque-meningioma (EPM) is characterized by its flat growth along the bony contour. It accounts for 2-9% of all meningiomas. Very few grade II or III EPM cases were reported. Surgical resection of sphenoid wing EPM is especially challenging as the tumour tends to invade the cavernous sinus, and/or the orbit, and their neurovascular structures. Consequently, tumours in such locations have a higher rate of recurrence. We report the clinical course and management of a patient suffering a second recurrence of grade II EPM. The clinical course of grade II EPM, and the management of multiple recurrences of EPM are scarcely reported in the literature.Case presentationA 53-year-old male with a history of three previous surgeries for EPM presented with decreased vision in the right eye. Brain magnetic resonance imaging (MRI) showed progression of a sphenoid wing meningioma invading the left optic nerve, indicating a second recurrence of the tumour.Clinical discussionWe reviewed the literature discussing the clinical course of grade II EPM, and cases suffering multiple recurrences. Only a few cases were found with varying clinical course and management. In our case, surgical intervention was necessary to save the patient's vision. A modified orbitozygomatic craniotomy was performed. A small residual tumour invading the cavernous sinus was left for treatment with stereotactic radiosurgery.ConclusionSphenoid wing EPM is challenging pathology to manage, especially grade II tumours which are rarely encountered. Multimodality treatment with surgery and radiotherapy offers EPM patients the best chance of treatment.
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Project description:BackgroundAdult-onset colonic intussusception is a rarely encountered condition that leads to large intestinal obstruction with time. Patients often present with a variety of symptoms that are non-specific making it challenging to arrive at a definitive diagnosis. This is worrying as diagnostic delay could lead to a significant increase in morbidity and mortality. We wish to present and describe a case of an atypical endoscopic finding of colocolic intussusception secondary to ascending colon cancer.Case descriptionSixty-seven-year-old lady was referred for 1 month's duration of passing melenic stools with mucus followed by a week's complain of hematochezia. Clinical examination and other relevant blood results were unremarkable except for iron deficiency anemia. Initial colonoscopy revealed a large mass within the splenic flexure with inconclusive biopsies. A more detailed colonoscopy repeated the following day revealed a massive, black-to-yellowish lesion within the splenic flexure with no viable mucosa seen. Colonic bezoar was initially suspected, however various endoscopic retrieval methods proved futile. Switching to a slimmer diagnostic gastroscope, the colon was carefully negotiated until a large ulcer was found within the ascending colon, adjacent to the mass' origin. An emergency CT abdomen and subsequently extended right hemicolectomy performed revealed a colocolic intussusception with sealed perforation secondary to an ascending colonic mass acting as an intussusceptum. Histopathology evaluation confirmed an ascending colon adenocarcinoma (pT2N0M0) amidst a background of extensive ischemic changes.ConclusionsEndoscopic descriptions of colonic intussusception are unusual given their rarity. Furthermore, these lesions can mimic a colonic bezoar as a result of fecal accretion and this can ultimately lead to false diagnostic and therapeutic decisions. In such instances, clarification with a CT scan before management decision can potentially avert unnecessary endoscopic intervention and complications.
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