Adenoid cystic carcinoma with a leptomeningeal dissemination in a ‘wash’ pattern

2021 ◽  
Vol 14 (12) ◽  
pp. e247155
Author(s):  
Juan Daniel Prieto Cuadra ◽  
Maria Teresa Dawid de Vera ◽  
Eva García Carrasco ◽  
M Isabel Hierro Martín

A 39-year-old woman was referred to the neurology department due to headache, instability and difficulty walking for 5 months. Several ancillary tests were performed. The blood test showed leucocytosis and the cerebrospinal fluid revealed an increased total protein and glucose consumption. Other infections or autoimmune causes were excluded. The MRI showed non-specific brain and spinal cord lesions. Given the findings described, a differential diagnosis between granulomatous meningoencephalitis and primary tumour or metastasis was proposed. Empirical treatment with tuberculostatic agents and corticosteroids was started. The neurological state of the patient worsened, she fell into a non-responsive coma and died in few days. The clinical autopsy performed revealed an adenoid cystic carcinoma with involvement of the central nervous system that developed leptomeningeal dissemination along the spinal cord in a fluid ‘wash’ pattern.

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jian Chen ◽  
Jingfang Mao ◽  
Ningyi Ma ◽  
Kai-Liang Wu ◽  
Jiade Lu ◽  
...  

Abstract Background Tracheobronchial adenoid cystic carcinoma (TACC) is a rare tumour. About one-third of patients miss their chance of surgery or complete resection as it is mostly detected in the advanced stage; hence, photon radiotherapy (RT) is used. However, the outcomes of photon RT remain unsatisfactory. Carbon ion radiotherapy (CIRT) is thought to improve the therapeutic gain ratio; however, the outcomes of CIRT in TACC are unclear. Therefore, we aimed to assess the effects and toxicities of CIRT in patients with TACC. Methods The inclusion criteria were as follows: 1) age 18–80 years; 2) Eastern Cooperative Oncology Group Performance Status 0–2; 3) histologically confirmed TACC; 4) stage III–IV disease; 5) visible primary tumour; and 6) no previous RT history. The planned prescription doses of CIRT were 66–72.6 GyE/22–23 fractions. The rates of overall survival (OS), local control (LC), and progression-free survival (PFS) were calculated using the Kaplan-Meier method. Treatment-induced toxicities and tumour response were scored according to the Common Terminology Criteria for Adverse Events and Response Evaluation Criteria in Solid Tumors, respectively. Results Eighteen patients with a median age of 48 (range 30–73) years were enrolled. The median follow-up time was 20.7 (range 5.8–44.1) months. The overall response rate was 88.2%. Five patients developed lung metastasis after 12.2–41.0 months and one of them experienced local recurrence at 31.9 months after CIRT. The rates of 2-year OS, LC, and PFS were 100, 100, and 61.4%, respectively. Except for one patient who experienced grade 4 tracheal stenosis, which was relieved after stent implantation, no other ≥3 grade toxicities were observed. Conclusions CIRT might be safe and effective in the management of TACC based on a short observation period. Further studies with more cases and longer observation are warranted.


2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Song Cao ◽  
Daniel W. Fisher ◽  
Guadalupe Rodriguez ◽  
Tian Yu ◽  
Hongxin Dong

Abstract Background The role of microglia in Alzheimer’s disease (AD) pathogenesis is becoming increasingly important, as activation of these cell types likely contributes to both pathological and protective processes associated with all phases of the disease. During early AD pathogenesis, one of the first areas of degeneration is the locus coeruleus (LC), which provides broad innervation of the central nervous system and facilitates norepinephrine (NE) transmission. Though the LC-NE is likely to influence microglial dynamics, it is unclear how these systems change with AD compared to otherwise healthy aging. Methods In this study, we evaluated the dynamic changes of neuroinflammation and neurodegeneration in the LC-NE system in the brain and spinal cord of APP/PS1 mice and aged WT mice using immunofluorescence and ELISA. Results Our results demonstrated increased expression of inflammatory cytokines and microglial activation observed in the cortex, hippocampus, and spinal cord of APP/PS1 compared to WT mice. LC-NE neuron and fiber loss as well as reduced norepinephrine transporter (NET) expression was more evident in APP/PS1 mice, although NE levels were similar between 12-month-old APP/PS1 and WT mice. Notably, the degree of microglial activation, LC-NE nerve fiber loss, and NET reduction in the brain and spinal cord were more severe in 12-month-old APP/PS1 compared to 12- and 24-month-old WT mice. Conclusion These results suggest that elevated neuroinflammation and microglial activation in the brain and spinal cord of APP/PS1 mice correlate with significant degeneration of the LC-NE system.


Neurosurgery ◽  
1991 ◽  
Vol 29 (4) ◽  
pp. 575-579 ◽  
Author(s):  
Marc G. Reyes ◽  
Fayez M. Homsi ◽  
Larry W. McDonald ◽  
Roberta P. Glick

Abstract In this study, we compared the suitability and accuracy of imprints, smears, and frozen sections of suspected brain and spinal cord tumors of 150 patients. Eighty-six percent of the imprints, 91% of the smears, and 99% of the frozen sections were suitable for interpretation. Among the suitable preparations, 82% of the imprints, 92% of the smears, and 99% of the frozen sections agreed with our diagnosis on paraffin sections. Although frozen sections were clearly more accurate than imprints and smears, the latter two provided diagnoses in patients with acquired immunodeficiency syndrome where frozen sections were not done to avoid contaminating our cryostat and in a patient with an epidermoid cyst of the middle fossa that could not be adequately frozen sectioned. Our study shows that imprints and smears complement frozen sections in the intraoperative diagnosis of tumors of the central nervous system.


2008 ◽  
Vol 2 (1) ◽  
Author(s):  
Juliette Thariat ◽  
Laure S. Fournier ◽  
Cécile Badoual ◽  
Pierre-Yves Marcy ◽  
Martin Housset

2020 ◽  
Vol 73 (8) ◽  
pp. 476-482 ◽  
Author(s):  
Thomas J W Klein Nulent ◽  
Robert J J van Es ◽  
Matthijs H Valstar ◽  
Ludwig E Smeele ◽  
Laura A Smit ◽  
...  

AimTreatment options for head and neck adenoid cystic carcinoma (AdCC) are limited in advanced disease. Chemokine receptor type 4 (CXCR4) is present in various tumour types, including AdCC. Upregulation is associated with tumour recurrence and metastasis. New CXCR4-specific diagnostic and therapeutic target agents have recently been available. This study aimed to analyse CXCR4 expression in a cohort of primary head and neck AdCC.MethodsAfter histopathological revision, tumour tissues of 73 consecutive patients with AdCC over 1990–2016 were sampled on a tissue microarray. Slides were immunohistochemically stained for CXCR4 and semiquantitatively scored. Associations between protein expression and cliniopathological parameters were tested. HRs were calculated using a Cox proportional hazard model.ResultsSixty-six tumours could be analysed. CXCR4 expression was present in 81% of the tumours with a median of 29% (IQR 1–70) positive cells. Expression was univariately correlated to perineural growth (Spearman ρ .26, p=0.04) and bone invasion (Spearman ρ .32, p=0.01), but not with tumour grade.CXCR4 expression in the primary tumour was significantly higher in tumours that recurred as compared with those that did not recur (median 60%, IQR 33–72 vs 12%, IQR 1–70, Kruskal-Wallis p=0.01). After dichotomisation, >25% of CXCR4 expressions proved an independent prognosticator for a reduced recurrence-free survival (RFS) (HR 7.2, 95% CI 1.5 to 72.4, p=0.04).ConclusionCXCR4 is expressed in the majority of primary AdCCs and independently correlated to worse RFS, suggesting CXCR4 as a target for imaging and therapy purposes in patients with advanced AdCC.


1930 ◽  
Vol 51 (6) ◽  
pp. 889-902 ◽  
Author(s):  
Jules Freund

1. Antibodies can be extracted from the brain and spinal cord of rabbits actively or passively immunized with typhoid bacilli. 2. The titers of the antibodies in the extracts of brain and cord depend upon the titer of the blood serum. In actively immunized rabbits the following numerical relationships exist between the titers of the serum and of these organ extracts: The ratio of the titer of the serum is to the titers of extract of brain and of the spinal cord about as 100 is to 0.8; the titer of the serum is to the titer of the cerebrospinal fluid as 100 is to 0.3. In passively immunized rabbits the titer of the serum is to the titer of brain and spinal-cord extract as 100 is to 0.7. 3. The antibodies recovered from the brain are not due to the presence of blood in it for perfusion of the brain does not reduce its antibody content appreciably. 4. Antibodies penetrate into the spinal fluid from the blood even in the absence of inflammation of the meninges. When the penetration is completed the following numerical relationship exists between the titer of the serum and that of the cerebrospinal fluid: 100 to 0.25. 5. The penetration into the cerebrospinal fluid of antibodies injected intravenously proceeds at a slow rate, being completed only several hours after the immune serum has been injected. The penetration of antibodies into the tissue of the brain occurs at a very rapid rate. It is completed within 15 minutes. 6. It is very unlikely that when the immune serum is injected intravenously the antibodies reach the brain tissue by way of the cerebrospinal fluid, for (1) the antibody titer of the cerebrospinal fluid is lower than that of the brain extract, and (2) antibodies penetrate faster into the tissue of the brain than into the cerebrospinal fluid.


1992 ◽  
Vol 106 (11) ◽  
pp. 1016-1020 ◽  
Author(s):  
Yuzo Yamamoto ◽  
Tetsuro Saka ◽  
Kazuo Makimoto ◽  
Hiroaki Takahashi

AbstractEight cases of adenoid cystic carcinoma were reviewed to determine whether and how the histological features of the tumour vary with the progress of the disease. The tumours were classified by their histological patterns as tubular, cribriform, trabecular or solid. The relative amount of each pattern seen in routine light microscopic sections was calculated histomorphometrically and compared in the primary tumour and recurrent and/or metastatic lesions in the same case. In the early stage, the tubular pattern predominated. Later, the highest percentage shifted to the cribriform, then to the trabecular and finally, in the late stage, to the solid pattern. There was no reverse direction of histological transformation from the solid to the tubular pattern. These results may help to explain previous reports that the tubular pattern usually represents a favourable prognosis, the solid pattern a poor prognosis and the cribriform pattern an intermediate prognosis.


Neurosurgery ◽  
2007 ◽  
Vol 61 (6) ◽  
pp. E1336-E1337 ◽  
Author(s):  
Daina Kashiwazaki ◽  
Kazutoshi Hida ◽  
Shunsuke Yano ◽  
Toshitaka Seki ◽  
Yoshinobu Iwasaki

Abstract OBJECTIVE Hemangiopericytomas, vascular tumors arising in soft tissue, are relatively rare in the central nervous system; they comprise less than 1% of all hemangiopericytomas. Central nervous system hemangiopericytomas occur primarily in the epidural space of the brain and spinal cord. There are no previous reports of subpial, extramedullary growing central nervous system hemangiopericytomas. CLINICAL PRESENTATION We document the first case of a subpial hemangiopericytoma with extramedullary growth in the thoracic spine. The patient was a 31-year-old man who developed progressively worsening left lower limb numbness that was followed by gait disturbance over the course of 4 months. INTERVENTION Magnetic resonance imaging revealed an intradural tumor at the T4–T6 level of the thoracic spine. Because the patient's symptoms progressed, he underwent resection of the tumor, which had arisen in the spinal cord subpially without attachment to the dura mater. CONCLUSION The pathological diagnosis was hemangiopericytoma. Differential diagnoses include hemangioblastoma, meningioma, schwannoma, and solitary fibrous tumor, the clinical course and prognosis of which are different from hemangiopericytoma. Our experience indicates that hemangiopericytomas can occur as intradural tumors arising from the subpial portion.


2015 ◽  
Vol 23 (3) ◽  
pp. 290-293 ◽  
Author(s):  
Ian Peeters ◽  
Jan W. Casselman ◽  
Stefaan J. Vandecasteele ◽  
Alexander Janssen ◽  
Bart Regaert ◽  
...  

Nocardiosis of the central nervous system is a challenging and difficult diagnosis for the clinician. The combination of infections of the brain and spinal cord is even more rare. The authors report on a patient with multiple lesions in the brainstem and cervical spinal cord. This 81-year-old immunocompetent woman presented with symptoms of progressive walking difficulty and ataxia. The results of an extensive workup with laboratory investigation, MRI, lumbar puncture, positron emission tomography (PET), and bone marrow biopsy remained inconclusive. Only after an open biopsy of a cervical lesion by an anterior approach through a partial central corpectomy of the cervical spine, was the diagnosis of nocardiosis made, allowing for specific antibiotic treatment.


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