Extracranial Mass Research Articles

Intracranial lipoma associated with a subcutaneous lipoma

Intracranial lipomas are rare, frequently asymptomatic, congenital malformations. They are most commonly located in the pericallosal region and are often detected incidentally during neuroimaging studies or postmortem examinations. While other associated brain malformations, most notably callosal agenesis, are frequently reported, association with a subcutaneous scalp lipoma is extremely rare. We present a case of pericallosal lipoma associated with callosal agenesis and subcutaneous lipoma over the anterior fontanelle in a 6-month-old female infant who had excision of only the extracranial mass and has remained asymptomatic from the intracranial mass for the 3 years of follow up.

"Abducens Nerve and Facial Nerve Palsy in the Setting of an Extracranial Mass and Vascular Disease"

Francis Demiraj* and Abbas Abidi Author Affiliations Kiran C Patel College of Osteopathic Medicine, Nova Southeastern University, USA Received: May 16, 2021 | Published: May 25, 2022 Corresponding author: Francis Demiraj, Kiran C Patel College of Osteopathic Medicine, Nova Southeastern University, Davie, FL 33314, USA DOI: 10.26717/BJSTR.2022.44.007003

Fatal high-grade skull osteosarcoma 30 years following radiotherapy for Cushing's disease.

SummaryCushing’s disease is a rare disorder characterised by excessive cortisol production as a consequence of a corticotroph pituitary tumour. While the primary treatment is surgical resection, post-operative radiation therapy may be used in cases of ongoing inadequate hormonal control or residual or progressive structural disease. Despite improved outcomes, radiotherapy for pituitary tumours is associated with hypopituitarism, visual deficits and, rarely, secondary malignancies. We describe an unusual case of a 67-year-old female with presumed Cushing’s disease diagnosed at the age of 37, treated with transsphenoidal resection of a pituitary tumour with post-operative external beam radiotherapy (EBRT), ketoconazole for steroidogenesis inhibition, and finally bilateral adrenalectomy for refractory disease. She presented 30 years after her treatment with a witnessed generalised tonic-clonic seizure. Radiological investigations confirmed an extracranial mass infiltrating through the temporal bone and into brain parenchyma. Due to recurrent generalised seizures, the patient was intubated and commenced on dexamethasone and anti-epileptic therapy. Resection of the tumour revealed a high-grade osteoblastic osteosarcoma. Unfortunately, the patient deteriorated in intensive care and suffered a fatal cardiac arrest following a likely aspiration event. We describe the risk factors, prevalence and treatment of radiation-induced osteosarcoma, an exceedingly rare and late complication of pituitary irradiation. To our knowledge, this is the longest reported latency period between pituitary irradiation and the development of an osteosarcoma of the skull.Learning points:Cushing’s disease is treated with transsphenoidal resection as first-line therapy, with radiotherapy used in cases of incomplete resection, disease recurrence or persistent hypercortisolism.The most common long-term adverse outcome of pituitary tumour irradiation is hypopituitarism occurring in 30–60% of patients at 10 years, and less commonly, vision loss and oculomotor nerve palsies, radiation-induced brain tumours and sarcomas.Currently proposed characteristics of radiation-induced osteosarcomas include: the finding of a different histological type to the primary tumour, has developed within or adjacent to the path of the radiation beam, and a latency period of at least 3 years.Treatment of osteosarcoma of the skull include complete surgical excision, followed by systemic chemotherapy and/or radiotherapy.Overall prognosis in radiation-induced sarcoma of bone is poor.Newer techniques such as stereotactic radiosurgery may reduce the incidence of radiation-induced malignancies.

Schwannomas of Uncommon Peripheral Locations: Analysis of Imaging Findings of 21 Cases.

Schwannomas are benign slow-growing tumors most often associated with the cranial nerves. Schwannomas often originate from the eighth cranial nerve. They may also originate from the peripheral nervous system of the neck and extremities. However extracranial peripheral schwannomas are considered a rare entity. The knowledge of rare localizations and typical imaging findings will lead to a successfulradiological diagnosis. Therefore, in this study, we present the clinical findings and MRI characteristics of schwannomas with a rare localization involving the peripheral, lower and upper extremity and intramuscular regions. The hospital database was screened for patients with an extracranial soft tissue mass. Twenty-one cases of schwannomas were found in rare localization. We analyzed the MR images of these patients retrospectively. The MR images were evaluated in terms of tumor location, signal intensity, and enhancement pattern. The histological examination of all the patients confirmed the diagnosis of schwannoma. In 21 patients, the schwannomas were peripheral, localized to upper (n = 6) and lower extremities (n = 11). The remaining four patients had intramuscular schwannomas. The patients diagnosed with intramuscular schwannomas had schwannomas in sternocleidomastoid, gastrocnemius, triceps muscle and lateral wall of the abdomen. The average long-axis diameter of the tumor was 27.7 mm and the average short-axis diameter was 16.4 mm. The contrast pattern was diffused in eight tumors and peripheral in 13. In this study, we present clinical findings and MRI characteristics of schwannomas with a rare localization involving the peripheral, lower and upper extremity and intramuscular regions.

The Clinical Importance of the Prenatal Diagnosis of Fetal Scalp Hemangioma

Scalp hemangioma is a rare disease of the extracranial mass in the fetus. Here, we demonstrate a case of fetal scalp hemangioma as evaluated by prenatal ultrasonography and magnetic resonance image (MRI). In this case, a fetal scalp mass measuring 6.3×3.7 cm was found at 32 weeks of gestation by ultrasonography. The possibility of fetal hemangioma was considered through 4D power Doppler ultrasonography and MRI. At 36+6 weeks of gestation, cesarean section was performed in order to avoid possible traumatic injury to the fetal scalp mass. Postnatally, the baby was diagnosed with scalp hemangioma, which was then treated with beta blockers. It is important to diagnose hemangioma in the prenatal stage, because such an early diagnosis can help plan the method and timing of delivery so as to reduce any risk of traumatic damage to the tumor. In addition, it can aid the prognosis and treatment of the neonate.

Three-dimensional ultrasound in evaluation of fetal brain anomalies

Background and objective: Prenatal diagnosis of central nervous system anomalies by two-dimensional sonography is challenging because of difficulties in obtaining complete visualization of the fetal brain during routine examinations. Three-dimensional sonography has been introduced as a tool for studying the fetal central nervous system because of its ability to facilitate examinations of the fetal brain. This study aimed to visualize an intracranial structure of the fetal central nervous system anomaly using transabdominal three-dimensional sonography. Methods: A prospective cross–sectional (observational) study in which three-dimensional ultrasound examination was performed in 82 patients between 12 and 38 weeks of gestation suspected to have fetal brain malformation detected by conventional two-dimensional ultrasound. Each anomaly was reviewed again to determine whether three-dimensional ultrasound data were advantageous compared to two-dimensional ultrasound. Results: Three-dimensional images provided additional information in 43 (52.4%) of cases including extracerebral anomalies. The three-dimensional ultrasound was advantageous in evaluating the encephaloceles (10 of 43 cases) in that the exact location of the extracranial mass and the amount of extracranial tissue in the encephalocele was better defined with the simultaneous display of three orthogonal planes images that could not be obtained with two-dimensional ultrasound were seen with three-dimensional ultrasound. Conclusion: Three-dimensional ultrasound is an excellent adjunctive tool to two-dimensional ultrasound in the evaluation of fetal anomalies

Magnetic resonance imaging features of esthesioneuroblastoma in three dogs and one cat.

Esthesioneuroblastoma is a rare malignant intranasal tumor that originates from the olfactory neuroepithelium of the upper nasal cavity, and can destroy the cribriform plate and expand into the neurocranium. Descriptions of the magnetic resonance features of esthesioneuroblastomas in animals are scarce. The objectives of this study were to report the magnetic resonance imaging features of esthesioneuroblastomas in order to determine distinct imaging characteristics that may help distinguish it from other intracranial tumor types. Magnetic resonance images of four patients with confirmed esthesioneuroblastomas were reviewed and compared with previously reported cases. The esthesioneuroblastomas appeared as oval-shaped, solitary lesions in the caudal nasal cavity that caused osteolysis of the cribriform plate and extended into the brain in all cases. Signal intensity was variable. Contrast enhancement was mild and varied from homogeneous to heterogeneous. A peripheral cystic component was found in two patients and was reported in only one previous case. Mass effect and white matter edema were marked to severe. Osteolysis of facial bones and extension into the facial soft tissues or retrobulbar space were not present in any of the cases, although this has been reported in the literature. A definitive diagnosis of esthesioneuroblastoma based on signal intensity or contrast behavior was not possible. Nevertheless, the presence of a mass in the caudal nasal cavity with extension into the neurocranium seems to be a feature highly suspicious of esthesioneuroblastoma. In contrast to other extra-cranial lesions, the extra-cranial mass was relatively small and destruction of facial bones seems to be rare.

DIAGNÓSTICO ECOGRÁFICO PRENATAL DE ENCEFALOCELE FRONTAL: A PROPÓSITO DE UN CASO

Se reporta un caso de encefalocele frontal, rara presentación de los defectos del tubo neural. Fue diagnosticado por ultrasonido a las 31 semanas de edad gestacional. Se describe las características ecográficas del encefalocele (masa extracraneal, ventriculomegalia y defecto óseo del cráneo) y se considera algunas entidades que pueden simular imágenes de encefalocele frontal para su diagnostico diferencial, como son glioma óptico hemangioma, teratoma. Se realizó estudios para determinar malformaciones asociadas, los cuales fueron negativos.

MB-07 * PRIMARY EWING SARCOMA OF THE SCALP WITH APPARENT EPIDURAL EXTENSION: CASE ILLUSTRATION

INTRODUCTION: Ewing sarcoma is an aggressive, rapidly growing, malignant tumor originating from postganglionic parasympathetic neural crest cells, most commonly originating in the pelvis and long bones. We describe a rare case of primary Ewing sarcoma of the scalp, in which imaging was suggestive of intracranial extension not seen on operative exploration. CASE REPORT: An 11 year old boy with a rapidly growing subcutaneous mass on the forehead was diagnosed with Ewing sarcoma by needle biopsy. Brain CT and MRI showed an extracranial mass with a smaller corresponding epidural lesion. Metastatic evaluation was negative. The patient was treated with chemotherapy to shrink the lesion, followed by surgical resection of the residual mass. In surgery, only partial-thickness erosion of the extracranial tumor mass through the bone was observed. The involved area of skull with margins was removed as a single bone flap. The inner table of the bone and epidural space appeared grossly normal, and there was no subdural mass on ultrasound. The patient subsequently completed further chemotherapy and radiation, and has remained disease-free. No residual or recurrent abnormal tissue has been seen on surveillance imaging. DISCUSSION: Ewing sarcoma of the scalp is a rarely reported disease entity, relatively less common than primary disease of the skull. Good outcomes have been reported when wide local excision is carried out. The phenomenon of radiographic epidural pseudoextension has not previously been described and does not seem to have adversely affected outcome in this case. CONCLUSIONS: MRI may overestimate extent of disease in cases adjacent to the epidural space. In cases of localized disease, wide local excision combined with chemotherapy and radiation can offer significant progression-free survival.

A Rare Case Report of Malignant Meningioma

Aims & Objectives: Atypical and malignant meningiomas are uncommon tumors with aggressive behavior and higher mortality, morbidity, and recurrence compared with benign tumors. We report a case report of malignant meningioma. Materials & Methods: A 53 year old male patient, presented with history large ulcerative swelling over the scalp for the past 5 years, with complaints of altered and aggressive behaviour and headache. Patient gives history of operation for benign meningioma 4 years back.Patient underwent MRI & CT Brain plain and contrast. Result: Patient underwent MRI & CT Brain plain and contrast, showed a large heterogeneously enhancing extraxial mass lesion with irregular margins ,significant extracalvarial spread and mushrooming into the brain parenchyma in the temporal & parietal regions. The lesion showed intratumoral cysts within it. On MRS significant choline,lipid and lactate peaks with near total dampening of NAA, Cr , MI peaks. The mean ADC values were (0.5 0.8 × 10−3 mm2/s) was suggesting a high grade cellular lesion. Conclusion: Anaplastic or malignant meningioma (MMen) corresponds histologically to WHO grade III. Frankly malignant meningiomas are rare, representing only 1-3% of all meningiomas. MMens have a striking male predominance. The imaging triad of extracranial mass, osteolysis, and “mushrooming” intracranial tumor is present in most but not all cases of Malignant meningiomas. Key-words: Malignant meningioma, mushrooming,extra-axial

Anaplastic hemangiopericytoma manifesting as a rapidly enlarging extracranial mass lesion

We, herein, present a patient with a recurrent anaplastic hemanigiopericytoma manifesting as a rapidly enlarging extracranial mass lesion, which was revealed by pathological and intraoperative findings. In practice, this case highlights the mandatory need for a careful long-term follow-up for patients with hemangiopericytoma, since recurrence with a greater degree of malignancy can develop following an extended disease-free interval, as such knowledge will be helpful for planning the optimal surgical procedures.

Primary Non-Hodgkin's Lymphoma of the Scalp and Cranial Vault

Primary Non-Hodgkin's Lymphoma of the cranial scalp and skull vault is a rare disease. We are describing a case of the same in a 50-year-old man. He was presented with a diffuse swelling in the left side scalp since 4 months of duration and progressively enlarging in size. On local Examination of the scalp, there was a diffuse swelling in the left parietal and occipital region of scalp. Imaging showed diffuse infiltration of the skull vault with extracranial soft tissue masses. Further investigations with CT scan chest, abdomen, and pelvis did not reveal any other evidence of systemic lymphoma. Biopsy of one of the scalp masses showed a small to intermediate cell B-cell lymphoma. Other nine previously reported cases of primary skull vault lymphoma were reviewed.

Undifferentiated Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) of the Head in a Dog

A six year-old intact female miniature poodle was presented with a soft mass of the forehead region. Computed tomography identified generalized frontal bone loss and a large extracranial mass, which had a low attenuation area of hemorrhagic necrosis with septation and enhancement of solid components. In magnetic resonance imaging, the mass was isointense in T1-weighted images except its fluid parts and hyperintense in T2-weighted images with lobulated by low-signal septa. Surgery was performed to remove the mass, and histopathologic examination revealed that the mass was consistent with undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma). The dog died from the rapidly recurrent mass and severe pulmonary metastasis.

Multifocal primary lymphoma of the cranial vault in a nonimmnunocompromised adolescent

Primary non-Hodgkin lymphoma (NHL) of the skull is extremely rare. The authors report a case in a 19-year-old boy who presented with a progressively increasing scalp mass on background of generalized headache of 6-months duration. Imaging showed multifocal skull vault lesion with intra- and extra-cranial soft tissue masses, causing permeative destruction of underlying skull bone. Further investigation failed to identify any other evidence of systemic lymphoma. Histopathology examination of superficial scalp mass showed low grade non-Hodgkin B cell lymphoma. The precise anatomical location and multifocal nature of the neoplasm occurring in young nonimmunocompromised individual makes this case clinically and radiologically unique.

Possible Role of Matrix Metalloproteinase in Osteolytic Intracranial Meningiomas

Abnormalities of the bone are frequently encountered in patients with meningioma, and hyperostosis and endostosis are common bone alterations in these tumors. Extensive bony destruction is very unusual in patients with meningioma. We report six cases of intracranial meningioma associated with an osteolytic lesion of the skull and discuss the underlying mechanisms that may be responsible for bone destruction in patients with meningioma. Six patients were classified into three groups, severe, moderate and mild, according to the degree of osteolytic bony destruction. The tumor was classified as intracranial or extracranial, depending on its location. We investigated the potential role of matrix metalloproteinase (MMP) in meningioma-associated osteolysis. The levels of MMP expression were determined by gelatin zymography, reverse transcription-quantitative PCR analysis (RT-PCR) and immunohistochemical analysis. Complete surgical removal of the lesion was performed in each patient. Histological examination revealed benign meningioma in four cases, and two cases of atypical meningioma. Patients did not have a poor prognosis except one case of recurred atypical meningioma. Gelatin zymography and RT-PCR detected high levels of MMP-2 in almost all extracranial masses in comparison with the intracranial masses and MMP-9 in two. There was no difference in the severity of bone destruction. Immunohistochemical analysis revealed MMP-2 expression in the vicinity of the bone destruction, and a few MMP-9-positive stainings were observed. Osteolysis of the skull in patients with meningiomas might not be indicative of malignant pathological features and poor prognosis. Invasion to the extracranial portion and osteolysis might be associated with MMP-2 expression in meningioma.

Delayed sub-aponeurotic fluid collections in infancy: Three cases and a review of the literature

Background:Sub-aponeurotic fluid collections (SFCs) in the neonatal period are poorly described in the literature. We describe the occurrence, possible etiologies and treatment of sub-aponeurotic fluid collections following the neonatal period.Case Description:We present 3 cases of previously healthy children who developed soft, fluctuant, extracranial masses several weeks after birth. All 3 children were seen by a pediatric neurosurgeon after parents noticed scalp masses between 5 and 9 weeks of age. All 3 children were found to be otherwise healthy. Two of the children were born via C-section and 1 child was born vaginally. The vaginal delivery was described as difficult and utilized vacuum assist. Scalp electrodes were placed in all 3 children for intensive monitoring during labor. These children received plain skull x-rays to assess for abnormalities, and 2 of the children underwent a non-contrast brain CT scan to better characterize the fluid collection. Plain x-rays and CT scans showed no abnormalities of the skull or ventricles. In both patients who underwent a CT scan, a soft tissue prominence was noted with a Hounsfield unit similar to water. All cases resolved between 5 and 9 weeks after initial presentation, with no long-term sequelae.Conclusion:SFCs presenting after the neonatal period are usually associated with benign soft tissue swellings. Use of fetal scalp electrodes has been shown to cause cerebrospinal fluid (CSF) leakage in the neonatal period and may result in delayed SFC. This condition is benign, and the recommended course of treatment is conservative management.

Occipital scalp hemangioma: Prenatal sonographic and magnetic resonance images

Fetal scalp hemangioma in the occipital region is extremely rare and its accurate diagnosis is essential for perinatal management. We present a case of occipital scalp hemangioma diagnosed by prenatal sonography and magnetic resonance imaging (MRI). An echogenic mass measuring 29 mm x 23 mm x 30 mm was found in the occipital region by sonography at 20 weeks of gestation. Color-flow Doppler sonography depicted vascularity only at the periphery of the mass. The MRI results indicated that the extracranial mass exhibited a heterogeneous appearance of predominantly hyperintensity on T1- and hypointensity on T2-weighted images without evidence of fat signals, suggesting a soft tissue lesion containing blood products. Based on sonographic and MRI findings, the fetus was diagnosed to have occipital scalp hemangioma. Postmortem examination revealed cavernous hemangioma with hemorrhage. The MRI was a valuable adjunct to sonography for prenatal evaluation of an occipital lesion.

Tumors in Pediatric Patients at Diffusion-weighted MR Imaging: Apparent Diffusion Coefficient and Tumor Cellularity

To prospectively assess whether there is a relationship between the apparent diffusion coefficient (ADC) and the histopathologic cell count and whether the ADC can enable differentiation of benign and malignant extracranial mass lesions in children. Institutional ethics approval and parent or guardian consent were obtained. Eleven malignant and eight benign lesions in 19 children (11 girls, eight boys; median age, 3.9 years; age range, 11 days to 15.5 years) who underwent magnetic resonance (MR) imaging of extracranial mass lesions-including a diffusion-weighted sequence (with b values 0, 500, and 1000 sec/mm(2))-and histopathologic analysis to prove findings were studied. The median ADC within each mass lesion was compared with the median cell count for 10 high-power microscopic fields in the specimen. The inverse regression between cell count and ADC was calculated. The difference in ADC between benign and malignant lesions was assessed by using the Mann-Whitney U test. There was an inverse relationship between ADC and cell count, expressed as ADC (in x10(-3) mm(2)/sec) = 0.56 + (66.2/cell count), with a relatively good fit to the observed data (analysis of variance R(2) = 0.541, F = 20.0, P < .001). The ADCs of benign lesions ranged from (0.84-2.83) x 10(-3) mm(2)/sec (median, 1.35 x 10(-3) mm(2)/sec; standard deviation, 0.68). The ADCs of malignant lesions ranged from (0.73-1.53) x 10(-3) mm(2)/sec (median, 1.00 x 10(-3) mm(2)/sec; standard deviation, 0.29). There was no significant difference in ADC between benign and malignant lesions (Mann-Whitney U = 22, P = .069). All highly cellular (>150 cells per high-power field) lesions had an ADC lower than 1.5 x 10(-3) mm(2)/sec. Although there is a significant relationship between cellularity and ADC, cell count probably is not the sole determinant of the ADC. Use of the ADC cannot enable accurate differentiation of malignant and benign lesions.

Temporomandibular Joint Cyst Presenting as Trigeminal Neuropathy and Middle Fossa Mass: Case Report

We describe the case of a temporomandibular joint cyst eroding into the middle fossa, initially causing compression of the trigeminal nerve and trigeminal neuropathy, and ultimately causing destruction of the second division of the nerve with facial numbness. A 50-year-old woman with rheumatoid arthritis on prednisone developed right-sided, initially lancinating facial pain that ultimately became dull in nature in the maxillary division of the trigeminal nerve. This change in pain was associated with the development of numbness in the same distribution. The facial pain gradually resolved, but the facial numbness persisted. Imaging revealed a heterogeneously enhancing mass in the middle fossa lateral to Meckel's cave. During surgery, the dura was elevated, and a smooth, firm lesion with several thinly encapsulated, cystic areas containing viscous, white fluid was identified. The mass had eroded through the floor of the middle cranial fossa. The thick, fibrous capsule was freed from the bone edges and was sectioned sharply extracranially. Pathological evaluation was consistent with a cyst originating from the temporomandibular joint. Extracranial mass lesions involving the middle fossa structures are uncommon and typically arise from the parotid gland. This case represents the first example of a middle fossa mass originating from an abnormal temporomandibular joint and a unique cause of trigeminal neuropathy. After excision, there has been no recurrence after 2 years of follow-up.

Aggressive intracranial and extra‐cranial epithelioid hemangioendothelioma: A case report and review of the literature

Epithelioid hemangioendothelioma is a rare tumor of intermediate malignancy. Twelve intracranial cases have been well documented. We report an intra- and extra-cranial single mass epithelioid hemangioendothelioma. We describe the case of a 27-year-old male who presented a left temporal and retroauricular pain and an intra and extra-cranial mass extending to the left infratemporal fossa. The neoplasm was isointense with gray matter in both T1 and T2-weighted images, showing marked nodular gadolinium enhancement. About 90% of the tumor was surgically removed. The neoplasm was fibrous, well defined and arose from the left temporal artery branches. Recovery was uneventful. The histological diagnosis was epithelioid hemangioendothelioma. Enhanced magnetic resonance scan was repeated at 3 and 6 months after surgery, showing an increasing mass volume extending to the surrounding tissues, including intracranially, infratemporal fossa and left orbit. The patient died 8 months after the initial diagnosis. Epithelioid hemangioendotheliomas can be very aggressive tumors, presenting initially at an intra- and extra-cranial location, due to its fast growth. From our review of the literature, they seem to have an equal gender distribution (M : F = 7 : 6) and tend to affect people under the age of 30 (10/13). We report a unique and extremely aggressive tumor. The rarity of reports and the continuous spectrum of differentiation, ranging from borderline to highly malignant are obstacles to identifying initial therapeutic protocols and the adjunctive therapy after surgery.