Linfoma difuso de células B grandes primario de clivus: Reporte de un caso

Resumen El clivus corresponde a una región de la fosa craneal posterior conformada por la unión del cuerpo del hueso esfenoidal y la porción basilar del hueso occipital, siendo sus lesiones poco frecuentes. Dentro de las lesiones clivales las más frecuentes son los cordomas (40%), condrosarcomas, adenomas ectópicos, linfomas, entre otros. Los linfomas no Hodgkin primario de hueso corresponden a una presentación extranodal, muy infrecuente en adultos, constituyendo sólo un 1% a 2% de estos en la base de cráneo. Se presentan principalmente con cefalea, síntomas B, diplopia y parestesias trigeminales. El origen primario clival es una manifestación aún más infrecuente, siendo su principal síntoma la cefalea. La sospecha debe ser alta requiriendo neuroimágenes, luego biopsia ya sea endoscópica o abierta. El manejo es con quimioterapia R-CHOP con un 67% de respuesta completa, 16% de detención de la progresión y 16% de progresión pese a tratamiento.

Abstract The clivus corresponds to a posterior cranial fossa region formed by the union of the body of the sphenoid bone and the basilar portion of the occipital bone, being its pathology very rare. Among the clival lesions, the most frequent are chordomas (40%), chondrosarcomas, ectopic adenomas, lymphomas, among others. Primary bone non-Hodgkin lymphomas correspond to an extranodal presentation, which is very infrequent in adults, while the skull base presentation corresponds only to 1% to 2%. They present mainly with headache, B symptoms, diplopia, and trigeminal paresthesia. The primary clival origin is an even more infrequent manifestation, with headache being its main symptom. The clinical suspicious must be high, requiring neuroimaging, then an endoscopic or open surgery biopsy. Management is standardized with R-CHOP chemotherapy with a 67% of complete response, 16% stop of progression and 16% progression despite treatment.

Mandibular metastasis as a presenting feature of a clival chordoma

Chordomas are rare tumors which arise from the embryological remnants of the notochord. These tumors can potentially arise from any region within the craniospinal axis and often clinically present as a diagnostic challenge. Chordomas are rare in patients younger than 40 years of age. The most common primary cancers that metastasize to the jaw bones are the ones originating from the breast, lung, kidney adrenal, colo-rectum, or prostate. Mandibular metastasis from a primary chordoma is an extremely rare occurrence with only five prior reports, three originating from primaries in the sacrococcygeal region, one from a lumbar spine primary and the other from a primary arising from the spheno-occipital region. A literature review did not reveal any prior reports of mandibular metastasis at presentation from a clival chordoma. We possibly report the first case of such an unusual clinical scenario in a 7-year-old male child and further discuss the evaluation and management of these rare tumors

Macroprolactinoma clival ectópico: una forma rara de presentación de adenoma hipofisario / Ectopic clival macroprolactinoma: a rare presentation of pituitary adenoma

Los adenomas pituitarios son los tumores hipofisarios más frecuentes siendo una entidad rara cuando se trata de adenomas ectópicos, es decir, sin conexión con la glándula pituitaria. Se cree que derivan de células residuales del tracto de migración embriológico desde la bolsa de Rathke. Su presentación clínica es muy variable porque depende de la producción hormonal y del efecto masa en estructuras adyacentes. Generalmente suponen un reto diagnóstico debido a su baja frecuencia, la clínica variable de presentación y que no presentan características específicas en las pruebas de imagen. Generalmente el diagnóstico se realiza de manera retrospectiva tras la resección quirúrgica. Presentamos el caso de un varón de 56 años que se presentó con unos valores de prolactina de 6647.5 ng/ml (2.2-17.7) con clínica de hipogonadismo aislada que se resolvió con tratamiento médico sin precisar resección quirúrgica, con una disminución de la densidad radiológica y estabilización del tamaño y sin clínica compresiva ni alteración visual.

Pituitary adenomas are the most common hypophyseal tumors being a rare entity when they are ectopic, without connection to the pituitary gland. They are thought to arise from residual cells in the migration tract from Rathke´s pouch. Its clinical presentation is variable depending on the hormonal production and the pressure effect on adjacent structures. They usually are a diagnostic challenge due to their low frequency, wide range of clinical presentation and not showing specific features on imaging techniques. The diagnosis is made usually retrospectively after surgical resection. We report the case of a 56 years old male that presented with a prolactine value of 6647.5 ng/ml (2.2-17.7) and isolated hypogonadism symptoms that resolved with medical treatment without surgery, diminishing the radiological density and stabilizing the size without having compresive symptoms nor visual disturbances.

Plasmocitoma solitario intracraneal: una presentación inusual / Intracranial solitary plasmacytoma: an unusual presentation

Background: Intracranial solitary plasmacytoma is extremely rare and its location in the clivus is unusual; its clinical presentation is very varied and the diagnostic images are similar to the other tumors in skull-base. Case presentation: 47-year-old woman with initial diagnosis of pituitary macroadenoma, attended with headache, nausea, vomiting, diplopia in the right eye and paralysis of her abducent nerve. She was reoperated by transsphenoidal endoscopic approach, concluding in solitary clivus plasmacytoma by biopsy and immunohistochemistry. Conclusions: Clivus is an unusual location for intracranial plasmacytoma. Early diagnosis, complete study and careful follow-up are required due to its high progression to multiple myeloma(AU)

Introducción: El plasmocitoma solitario intracraneal es extremadamente raro y su localización en el clivus es inusual; su presentación clínica es muy variada y las imágenes diagnósticas son similares a las de otros tumores de la base del cráneo. Reporte de caso: Se presenta el caso de una mujer de 47 años con diagnóstico inicial de macroadenoma hipofisiario, que cursó con cefalea, náuseas, vómitos, diplopía en ojo derecho y parálisis de su nervio abducente. Fue reintervenida por vía transesfenoidal endoscópica, concluyendo en plasmocitoma solitario del clivus por biopsia e inmunohistoquímica. Conclusiones: El clivus es una localización inusual para plasmocitoma intracraneal, se requiere un diagnóstico precoz, estudio completo y seguimiento cuidadoso por su alta progresión a mieloma múltiple(AU)

Incidentally-Discovered Extraosseous Cystic Nasopharyngeal Chordoma in a Papillary Thyroid Cancer Patient

Skull base chordomas are rare, malignant tumors arising from primitive notochord remnants of the axial skeleton and comprise approximately 25–35% of all chordoma cases. Nasal endoscopy in previous case reports has characterized nasopharyngeal chordomas as firm, semi-translucent masses protruding from the posterior nasopharyngeal wall with a pink, “meaty” appearance. However, the nasopharyngeal chordoma in the present case had a soft, cystic appearance, unlike the tumors previously described. Herein, an unusual case of an incidentally discovered nasopharyngeal chordoma is reported in a patient with papillary thyroid cancer; the discovered chordoma had a benign cystic appearance with no abnormal positron emission tomography-computed tomography (PET-CT) uptake.

Evaluation of morphometric features of fossa navicularis using cone-beam computed tomography in a Turkish subpopulation

PURPOSE: Fossa navicularis is a bone defect in the clivus. Familiarity with this anatomical variant is important because it is close to vital anatomical structures in the base of the skull. The aim of this study was to determine the prevalence and morphometric properties of fossa navicularis within the clivus in a Turkish subpopulation using cone-beam computed tomography (CBCT). MATERIALS AND METHODS: A total of 168 CBCT scans (female: 96, male: 71) were evaluated. High-quality CBCT images of patients without a syndromic condition or a history of neurological disease or surgery were included in the study. The prevalence, depth, length, and width of the fossa navicularis were performed. RESULTS: The prevalence of fossa navicularis was 27.5% (n=46 patients). Sex was not associated with the depth, length, or width of the fossa navicularis (P>0.05). A significant positive correlation was found between age and length of the fossa navicularis (P>0.05). CONCLUSION: Fossa navicularis was found to be rare (27.5%). Anatomical variants of the skull base can also be clearly identified on CBCT images. The results of this study may be useful to radiologists, anatomists, and surgeons interested in the skull base.

Anatomy and clinical importance of the extracranial clivus and surrounding structures / Anatomía e importancia clínica del clivus extracraneal y las estructuras circundantes

The aim of this study was to reveal the clinical importance of measurements taken from the pharyngeal tubercle (PT) to various anatomical structures around the extracranial clivus. Twenty-six adult dry Anatolian skulls were examined. The extracranial clivus and PT were used as landmarks from which various distances were measured using a digital caliper accurate to 0.01 mm. The following mean distances from the PT were observed; foramen lacerum (FL) (L: 17.15 mm, R: 17.4 mm) ; medial external margin of the carotid canal (CC) (L: 26.7 mm, R: 27.5 mm); anterior tip of occipital condyle (OC) (L: 16.4 mm, R: 16.3 mm); anterior margin of foramen magnum (FM) (10.8 mm); foramen ovale (FO) (L: 25.9 mm, R: 29.1); medial margin of the jugular fossa (JF) (L: 25.4 mm, R: 25.7 mm); medial external margin of the hypoglossal canal (HC) (L: 20.0 mm, R: 19.9 mm). Mean bilateral distances were: LFO-RFO: 45.34 mm; LFL-RFL: 20.1 mm; LCC-RCC: 52.1 mm; LOC-ROC: 17.6 mm; LJF-RJF: 45.2 mm; LHC-RHC: 33.5 mm. The following mean distances were observed from the FM: FM-OC (L: 8.3 mm, R: 9.3 mm); FM-HC (L: 17.8 mm, R: 17.4 mm). Also the mean distance of OC-HC were observed (L: 11.7 mm, R: 11.4 mm). Present measurements suggest that the PT can be used as an anatomical landmark during surgery involving clival pathology. However, the anatomy and variations of the extracranial clivus and surrounding structures must be taken into consideration.

El objetivo de este estudio fue revelar la importancia clínica de las mediciones tomadas desde el tubérculo faríngeo (TF) a diversas estructuras anatómicas alrededor del clivus extracraneal. Veintiséis cráneos, secos adultos, de Anatolia, fueron examinados. El clivus extracraneal y TF se utilizaron como puntos de referencia a partir de los cuales se midieron varias distancias con un calibrador digital con una precisión de 0,01 mm. Se observaron las siguientes distancias medias del TF: foramen lacerum (FL) (L: 17.15 mm, R: 17,4 mm); margen externo medial del canal carotídeo (CC) (L: 26,7 mm, R: 27,5 mm); parte anterior del cóndilo occipital (OC) (L: 16,4 mm, R: 16,3 mm); margen anterior del foramen magnum (FM) (10,8 mm); foramen oval (FO) (L: 25,9 mm, R: 29,1); margen medial de la fosa yugular (FY) (L: 25,4 mm, R: 25,7 mm); margen medial del canal hipogloso (CH) (L: 20,0 mm, R: 19,9 mm). Las distancias bilaterales medias fueron: LFO-RFO: 45,34 mm; LFLRFL: 20,1 mm; LCC-RCC: 52,1 mm; LOC-ROC: 17,6 mm; LJFRJF: 45,2 mm; LHC-RHC: 33,5 mm. Se observaron las siguientes distancias medias de la FM: FM-OC (L: 8,3 mm, R: 9,3 mm); FMCH (L: 17,8 mm, R: 17,4 mm). También se observó la distancia media de OC-CH (L: 11,7 mm, R: 11,4 mm). Las mediciones actuales sugieren que el TF puede ser utilizado como un hito anatómico durante la cirugía que involucra la patología clival. Sin embargo, se deben tener en cuenta la anatomía y las variaciones del clivus extracraneal y las estructuras circundantes.

Reto neuroquirúrgico actual del cordoma cervical / Current neurosurgical challenge of the cervical chordoma

El cordoma es un tumor osteocartilaginoso raro, de lento crecimiento, con una tasa de incidencia global de 8,4 casos por cada 10 millones de habitantes. Comúnmente aparece en la quinta y sexta década de la vida, predomina en el sexo masculino. Se presenta un paciente masculino de 47 años de edad, con cervicobraquialgia, cuadriparesia y masa tumoral palpable en región anterolateral del cuello. El estudio de resonancia magnética demostró la presencia de una lesión retrofraríngea con destrucción vertebral y compresión extradural. Se decidió resección quirúrgica de la lesión. El diagnóstico histopatológico por inmunohistoquímica arrojó como resultado, un cordoma(AU)

Chordoma is a rare, slow-growing osteocartilaginous tumor with an overall incidence rate of 8.4 cases per 10 million inhabitants. Commonly appears in the fifth and sixth decade of life, predominates in the male sex. We present a 47-year-old male patient with cervicobrachialgia, quadriparesis and palpable tumor mass in the anterolateral region of the neck. The magnetic resonance study showed the presence of a retropharyngeal lesion with vertebral destruction and extradural compression. Surgical resection of the lesion was decided. The histopathological diagnosis by immunohistochemistry resulted in a chordoma(AU)

Sectional anatomy study of the clivus regoin and its three dimensional reconstruction / 局解手术学杂志

Objective To study the anatomical characteristics of sectional clivus region and to provide anatomical data for the operation of the clivus regoin.Methods Continuous thin sections(transverse position and coronal position)from two head specimens were performed with freezing milling technique.Observed the anatomical relationship and anatomical characteristics of the clivus region,and finished the three dimensional reconstruction based on the data of sections.Results Totally obtained 320 transverse sections and 232 coronary sections.The related structures were described in 4 typical sections,and three dimensional reconstruction of the clivus region were produced.Conclusion Combinationof section anatomy and three dimensional reconstruction can stereoscopically display the anatomical characters of the clivus re -gion.The three dimensional models could continuously and dynamically display the anatomic structures.

3-D simulation of posterior fossa reduction in Chiari I / Simulação em 3D da redução da fossa posterior no Chiari do tipo I

ABSTRACT We proposed a 3D model to evaluate the role of platybasia and clivus length in the development of Chiari I (CI). Using a computer aided design software, two DICOM files of a normal CT scan and MR were used to simulate different clivus lengths (CL) and also different basal angles (BA). The final posterior fossa volume (PFV) was obtained for each variation and the percentage of the volumetric change was acquired with the same method. The initial normal values of CL and BA were 35.65 mm and 112.66º respectively, with a total PFV of 209 ml. Ranging the CL from 34.65 to 29.65 – 24.65 – 19.65, there was a PFV decrease of 0.47% – 1.12% – 1.69%, respectively. Ranging the BA from 122.66º to 127.66º – 142.66º, the PFV decreased 0.69% – 3.23%, respectively. Our model highlights the importance of the basal angle and clivus length to the development of CI.

RESUMO No presente estudo, propusemos a criação de um modelo computacional em 3D com elaboração de software onde dois arquivos em formato DICOM com uma TC e RNM de crânio foram usados para simular diferentes mensurações na extensão do clivus (EC) e no ângulo basal (AB). O volume final da fossa posterior (VFP) foi obtido em cada variação, bem como a percentagem de volume alterada. O tamanho inicial da EC era de 35,65 mm e o do AB era de 112.66º, com um VFP de 209 ml. Variando a EC de 34,65 para 29,65 – 24.65 e 19.65, houve uma diminuição do VFP de 0.47%, 1.12% e 1.69%, respectivamente. Variando o AB de 122,66º para 127,66º e 142,66º, o VFP diminui para 0.69% e 3.23%, respectivamente. Nosso modelo enfatiza a importância da patogênese do aumento do AB e do encurtamento do clivus no desenvolvimento do Chiari I.

Fossa navicularis magna detection on cone-beam computed tomography

Herein, we report and discuss the detection of fossa navicularis magna, a close radiographic anatomic variant of canalis basilaris medianus of the basiocciput, as an incidental finding in cone-beam computed tomography (CBCT) imaging. The CBCT data of the patients in question were referred for the evaluation of implant sites and to rule out pathology in the maxilla and mandible. CBCT analysis showed osseous, notch-like defects on the inferior aspect of the clivus in all four cases. The appearance of fossa navicularis magna varied among the cases. In some, it was completely within the basiocciput and mimicked a small rounded, corticated, lytic defect, whereas it appeared as a notch in others. Fossa navicularis magna is an anatomical variant that occurs on the inferior aspect of the clivus. The pertinent literature on the anatomical variations occurring in this region was reviewed.

A Case of Aspergillus Clival Abscess Treated by Endoscopic Removal / 대한이비인후과학회지

Aspergillus clival abscess, especially without a sphenoid sinus lesion, is a very rare infection of central nervous system. In this report, we describe the successful treatment of a patient with aspergillus clival abscess. A 63-year-old man was referred to our hospital with the diagnosis of sphenoid mass lesion in magnetic resonance image accompanied by headache and diplopia. The computed tomography scan revealed a clival cystic lesion without connection with the sphenoid sinus. The patient underwent the endoscopic endonasal clival approach and histopathological examination revealed aspergillosis. Postoperatively, the patient was given IV amphotericin-B for 2 weeks and oral voriconazole for 3 months. After surgery, patient's headache and diplopia disappeared. Aspergillus abscess originating from the skull base is rare but has a high mortality rate. Histopathological confirmation via endonasal approach and intensive antifungal therapy should be started for a successful treatment.

Abordaje transmaxilar segmentado para resección de tumores del clivus: Reporte de un caso / Segmented trans-maxillary approach for clivus tumor resection: Case report

La compleja anatomía de las estructuras vitales de la base del cráneo dificulta mucho la resección quirúrgica de los tumores que afectan esta zona. El problema fundamental de los tumores que afectan a la base del cráneo es elegir el abordaje ideal. El desarrollo inicial de la cirugía de la base del cráneo fue producto de la colaboración entre la otorrinolaringología y la neurocirugía; la participación del cirujano maxilofacial ha sido un fenómeno relativamente reciente. En este caso se presenta un abordaje transmaxilar para acceso al clivus y eliminar el tumor.

The complex anatomy exhibited by the vital structures of the skull base hinders surgical resection of tumors present in that area. The main problem when facing tumors lodged in the skull base resides in choosing the most suitable approach. The initial development of skull base surgery was a product of the collaboration between otorhinolaryngology and neurosurgery techniques. The participation of maxillofacial surgeons in these events has been a relatively recent endeavor. In the present instance a case of trans-maxillary approach to access clivus and remove a tumor was presented.

Atypical Clival Chordoma in an Adolescent without Imaging Evidence of Bone Involvement

Clival chordoma is a rare primary bone tumour that arises from the remnant of the notochord and typically occurs in older adults. Upon imaging, the tumour can be seen arising from the clivus and causes clival destruction. This usually provides insight for a diagnosis. Here we present a case of a non-enhancing, pre-pontine mass that was hypointense on T1W and hyperintense on T2W in an adolescent. No clival bone erosion was observed. Based on the age group, imaging findings, and lack of clival erosion, a provisional diagnosis of epidermoid cyst was made and the tumour was resected. This patient was eventually diagnosed with a clival chordoma based on histopathological examination.

A Case of Endoscopic Removal of Clival Chordoma through Extended Transclival Approach / 대한이비인후과학회지

Clival chordoma is a rare malignant tumor which arises from the remnants of notochord. Although it is a slow-growing tumor that rarely metastasize to other organs, it is regarded as clinically malignant because of local invasiveness and high recurrence rate. Furthermore, because its location is critical and surgical resection is difficult, it has poor prognosis. Neurosurgeons have traditionally taken the lead in managing tumor, however, with the development of endoscopic techniques and wide spread of usage in skull base surgery, the role of ENT surgeon has become bigger recently. We report a recent case of recurrent clival chordoma successfully removed by endoscopic extended transclival approach.

Intradural Clival Chordoma: A Case Report

Clival chordoma is a rare intracranial neoplasm located in the clivus with bony extension and destruction. It is difficult to resect completely and generally has a poor prognosis. However, intradural clival chordomas have been reported with good surgical outcomes. We present a rare case of intradural chordoma and a review of the literature.

A Case of Giant Skull Base Chondroma

Intracranial chondroma is a rare benign tumor. Here, we present the case of a 29-year-old female who was afflicted with left eye blindness and ptosis. Brain computerized tomography and magnetic resonance imaging revealed the presence of a giant calcified mass accompanied by a solid mass in the middle and posterior fossa. A differential diagnosis regarding chordoma, chondrosarcoma, and other chondroid tumors based on radiologic information was inconclusive. The lesion was resected completely under a microscope using a combined pterional and subtemporal approach. The pathologic report confirmed the diagnosis of chondroma. No evidence of neurological worsening was observed. The tumor had a calcified mass with mature hyaline cartilage surrounded by a thick fibrous capsule. We dissected the periphery of the tumor mass and removed it via aspiration. It was readily distinguished from normal brain parenchymal tissue. The large calcified mass at the center of the tumor had relatively high vascularity, and a high-speed drill and various rongeurs were used to remove the tumor.

Traumatic retroclival epidural hematoma and unilateral occipital condylar fracture in an adult patient

Retroclival epidural hematoma is rare, with fewer than 30 cases reported in the literature. Almost all of the previously reported cases occurred in children, with only a few cases affecting adults. Among these adult patients, only two suffered from the associated occipital condyle fracture (OCF), which is another rare injury. The patient involved in this study suffered from retroclival epidural hematoma and unilateral occipital condyle fracture, as demonstrated on both CT and MRI scans. The patient also suffered from an associated right brachial plexus injury without any other neurological deficits, a condition that has not previously been reported. This paper reviews the underlying mechanisms of these rare injuries and seeks to form an understanding of them before proposing the mechanism of injury in the patient involved in the study

Fracturas del clivus / Clivus fractures

Las fracturas de base de cráneo se encuentran entre el 3.5 y el 24 por ciento de los pacientes con trauma craneoencefálico. Las fracturas del clivus, se consideran fracturas de la base craneana posterior y se clasifican en longitudinales, transversas y oblicuas. El diagnóstico de estas fracturas ha aumentado con el uso del TAC de cráneo. Anteriormente su diagnostico se realizaba con mayor frecuencia en autopsias. Las fracturas del clivus con gran frecuencia se asocian a lesión de estructuras neurológicas y vasculares vecinas, tienen un mal pronóstico neurológico aunque se han descrito casos asintomático de fracturas clivales. En este artículo se reportara nuestra experiencia con esta patología traumática y se hará una revisión de la literatura.

Skull base fractures are found in 3.5 to 24 percent of all traumatic brain injury. Clivus fracture is considered a posterior skull base fracture and is classified into longitudinal, transversal and oblique. The finding of these fractures had incremented with the use of CT scans. Previously the diagnosis of clival fractures was made mostly at autopsy studies. Clival fractures are associated with injuries to nervous and vascular structures in its vicinity. These fractures have a bad neurological prognosis although there are cases of asymptomatic patients with clival fractures in the scientific literature. In this article we will repot our experience with this traumatic pathology and we will review the literature.

Transoral needle procedures for upper cervical spine and clival lesions: technical note and indications / Procedimentos percutâneos transorais com agulha para lesões do clivo e da coluna cervical alta: indicações e nota técnica

Transoral needle procedures are minimally invasive techniques derived from the transoral (or transoropharyngeal) approach to the upper cervical spine and clival region. They are indicated for diagnostic procedures and vertebroplasty. These techniques are appropriated to access midline pathologies from the lower clivus to the C2-C3 disk. This article describes in a step by step manner, the technique and indications for needle biopsy and vertebroplasty in this region, discussing technical nuances.

Procedimentos transorais com agulhas são técnicas minimamente invasivas derivadas do acesso transoral (ou transorofaríngeo), utilizadas para cirurgias da coluna cervical superior e região do clivo. São geralmente indicados para procedimentos diagnósticos ou vertebroplastia, em lesões localizadas na linha média, do clivo inferior até o espaço discal C2-C3. No presente artigo, descrevem-se passo a passo a técnica e as indicações para biópsia de agulha e vertebroplastia nessa região, discutindo nuances técnicas.