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Meningeal Melanocytoma


Presentation

  • A case report of spinal meningeal melanocytoma with a dumbbell-shaped extension and its magnetic resonance imaging features is presented. To present a rare spinal tumor with pathologic and radiologic features.[ncbi.nlm.nih.gov]
  • The 17th case of an intracranial meningeal melanocytoma is presented in a 67-year old man. It is the 6th melanocytoma arising from the cavum Meckeli and the first presenting with seizures.[ncbi.nlm.nih.gov]
  • A unique case of a meningeal melanocytoma located in the pineal region is presented. This 48-year-old man presented with a round pineal region tumor that caused triventricular hydrocephalus and exhibited slow clinical progression.[ncbi.nlm.nih.gov]
  • Clinical presentation of patients with these tumors typically occurs in their fifth decade and women are affected twice as often as men.[ncbi.nlm.nih.gov]
  • Thus most reported cases of meningeal melanocytomas are located in the posterior fossa and the spinal cord, and presentation of a supratentorial tumor is rare. A 19-year-old man presented with a headache and seizure at our department.[ncbi.nlm.nih.gov]
Fecal Incontinence
  • The patient's presenting symptoms were characteristic of a large bifrontal lesion and included headaches, personality change, lethargy, and urinary and fecal incontinence.[ncbi.nlm.nih.gov]
Malocclusion
  • Here, we report a case of a 39-year-old Japanese man who had a history of pain and numbness on the left side of his face and malocclusion for 3 months before the initial visit.[ncbi.nlm.nih.gov]
Retinal Pigmentation
  • Bilateral naevus of Ota with choroidal melanoma and diffuse retinal pigmentation in a dark skinned person. Br J Ophthalmol. 2005;89:1529–45S. 46. Singh M, Kaur B, Annuar NM. Malignant melanoma of the choroid in a naevus of Ota.[docksci.com]
Macula
  • Jurgens I, Roca G, Sedo S, Pujol O, Berniell JA, Quinatana M (1994) Presumed melanocytoma of the macula. Arch Ophthalmol 112: 305–306 23.[slideheaven.com]
Subcutaneous Nodule
  • Radhadevi et al reported a 45-year-old male with malignant melanoma of the left orbit in association with nevus of Ota and MartínezPe nuela et al reported a 24-year-old man who presented with a subcutaneous nodule that had developed in the anterolateral[docksci.com]
Progressive Hearing Loss
  • Two women, aged 21 and 30 years, were admitted to the hospital 60 years apart: the first because of progressive paraplegia and the second because of slowly progressive hearing loss.[ncbi.nlm.nih.gov]
Irregular Menstruation
  • The patient presented with visual impairment and irregular menstruation with no other symptoms. Her general examination was otherwise unremarkable. Endocrine tests disclosed normal endocrine function except for slight hyperprolactinemia.[ncbi.nlm.nih.gov]
Urinary Retention
  • A 68-year-old female presented symptoms of urinary retention, loss of strength and lower limb dysesthesias of about 20 days’ evolution.[elsevier.es]
Meningism
  • Meningeal melanocytoma is an essentially benign melanotic tumor, derived from the melanocytes of the leptomeninges, and may occur anywhere in the cranial and spinal meninges.[ncbi.nlm.nih.gov]
  • Meningeal melanocytoma is a benign melanocytic tumor that originates most frequently from the melanocytes in the posterior fossa or along the spinal cord.[ncbi.nlm.nih.gov]
  • Meningeal melanocytomas are rare lesions usually found in the posterior fossa and upper cervical spine. The review of literature shows the variation in different studies.[ncbi.nlm.nih.gov]
  • Meningeal melanocytomas are rare benign pigmented tumors of meningeal melanocytes. Nevus of Ota is a melanocytic pigmentation of the sclera and the skin adjacent to the eye.[ncbi.nlm.nih.gov]
  • Histological examination revealed a typical meningeal melanocytoma as described by Limas and Tio in 1972. Ten additional cases of previously reported spinal meningeal melanocytomas are reviewed.[ncbi.nlm.nih.gov]
Tingling
  • Our patient experienced numbness and tingling in her left leg for 8 years, and low back pains with intermittent claudication for the previous 2 months. A histologically benign 20-mm tumour was totally resected. Radiation therapy was not given.[ncbi.nlm.nih.gov]
  • This patient presented clinically with paraparesis, tingling sensation and numbness of both lower extremities of 4 months duration. No mucocutaneous pigmented nevi were found.[ncbi.nlm.nih.gov]
Hyperreflexia
  • The neurologic examination revealed that the patient had left-sided lower extremity weakness of 4 , decreased sensation on the right side, and hyperreflexia in both legs. Department of Neuroradiology interpreted CT and MR imaging as meningiom.[ncbi.nlm.nih.gov]
  • A 79-year-old female presented with difficulty ambulating and was found to have weakness and hyperreflexia in the lower extremities. Magnetic resonance imaging (MRI) revealed a large T8-T9 intraspinal tumor.[ncbi.nlm.nih.gov]
Motor Symptoms
  • In the present study, a 58 year old lady presented with bilateral sensory and motor symptoms in the lower limbs.[ncbi.nlm.nih.gov]
Foster-Kennedy Syndrome
  • Fundoscopy showed left optic nerve atrophy and right papilledema consistent with Foster-Kennedy syndrome. Neurological exams were otherwise normal.[ncbi.nlm.nih.gov]

Workup

  • The histological workup following subtotal tumor resection of the initial tumor had revealed the typical features of a fibrous melanocytic meningioma without increased proliferation.[thejns.org]
  • It is important to differentiate primary melanin-containing lesions of the CNS from metastatic melanoma, because these lesions require a different patient workup and alternate therapeutic options.[pubs.rsna.org]
Hyperprolactinemia
  • Endocrine tests disclosed normal endocrine function except for slight hyperprolactinemia. Magnetic resonance imaging revealed a sellar lesion. The patient underwent a successful total transsphenoidal removal of the tumor without neurologic sequelae.[ncbi.nlm.nih.gov]

Treatment

  • The treatment of choice for SPMM is complete surgical resection via a transsphenoidal approach. For patients with a partially resected tumor, radiotherapy is an effective complementary treatment modality.[ncbi.nlm.nih.gov]
  • Complete tumor resection is considered the best treatment option, followed by incomplete resection with postoperative radiotherapy, but we could not deliver aggressive treatment in our patient because of hemorrhage from the tumor surface and his poor[ncbi.nlm.nih.gov]
  • Total resection is the best treatment, but gamma knife radiosurgery is effective for the residual tumor following partial resection.[ncbi.nlm.nih.gov]
  • They review the literature relevant to this topic and discuss the radiological and pathological features of this disease as well as its treatment options.[ncbi.nlm.nih.gov]
  • In conclusion, MMs are rare histologically benign tumors that can be cured by complete surgical resection alone, which should be the goal of the treatment.[ncbi.nlm.nih.gov]

Prognosis

  • Meningeal melanocytoma is a rare benign tumor with relatively good prognosis. However, local aggressive behavior of meningeal melanocytoma has been reported, especially in cases of incomplete surgical resection.[ncbi.nlm.nih.gov]
  • The prognosis of this tumor is not always favorable with occasional local recurrence. Total resection is the best treatment, but gamma knife radiosurgery is effective for the residual tumor following partial resection.[ncbi.nlm.nih.gov]
  • A brief review of literature pertaining to the radiological features, pathological findings, management and prognosis of this rare tumor is discussed.[ncbi.nlm.nih.gov]
  • In spite of benign biologic behavior of meningeal melanocytoma, the prognosis remains uncertain, because of the possible local recurrences.[ncbi.nlm.nih.gov]
  • Age of onset is 40 to 50 years old, mostly presenting a benign course of disease and the prognosis is good. Case reports of partial invasion or metastasis from lesions are even rarer.[ncbi.nlm.nih.gov]

Etiology

  • Etiology of Increased ICP Intracranial hypertension is defined as a sustained ( 5 min) elevation of ICP of 20 mmHg. Image : “MRI showing injury due to brain herniation in a patient with choriocarcinoma.” By Rocque BG, Başkaya MK (2008).[lecturio.com]
  • The -nal diagnosis was chronic intracerebral hemorrhage of unknown etiology with reactive changes of the surrounding brain tissue and perifocal extramedullary erythropoiesis.[docslide.com.br]
  • When no inflammatory, infectious or tumoral etiology or evidence of intracranial hypotension is present, a diagnosis of idiopathic pachymeningitis is considered, with possible dural calcifications.[em-consulte.com]
  • The lesion was excised and diagnosed as a malignant melanoma EP Conclusion TE D M AN U SC The etiology and pathogenesis of nevi of Ota and Ito are not known.[docksci.com]
  • Several theories for the etiology of melanotic schwannomas have been proposed, including the melanomatous transformation of neoplastic Schwann cells, phagocytosis of melanin by Schwann cells, and the simultaneous presence of two distinct neoplastic populations[pubs.rsna.org]

Epidemiology

  • Their epidemiological features, natural history and response to treatment remain poorly understood, even if, in the last 2 years, some reviews have been published about it.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Changes in glycosaminoglycans, which fill the cell free space in early embryologic development, may play an important role in the neural crest migration, leading to dermal melanosis.[17] Exact pathophysiology is not yet known.[docksci.com]

Prevention

  • Radiation therapy is important to prevent relapse of the tumor, especially in cases of incomplete surgical resection.[ncbi.nlm.nih.gov]
  • Radiation therapy is important to prevent relapse of the tumor when complete surgical resection is not possible. However the management of melanocytoma has no definite guidelines.[omicsonline.org]
  • This is helpful for the prevention of the impending complications.[lecturio.com]
  • Whenever total resection is not possible radiotherapy is essential to prevent recurrence.[docksci.com]
  • Hydrocephalus is usually treated with placement of a ventriculoperitoneal shunt, but a filter must be added to the apparatus to prevent spread in the rare event of malignant transformation (10).[ajnr.org]

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