Primary central nervous system ALK-negative anaplastic large cell lymphoma

Primary central nervous system ALK-negative anaplastic large cell lymphoma

see also Primary central nervous system ALK-positive anaplastic large cell lymphoma


Primary central nervous system anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL) is an extremely rare type of primary central nervous system lymphoma (PCNSL).

There are only nine cases reported in the literature to date, most of which have an overall survival time of no more than 8 months. Yuan et al. reported such a rare case that has a good outcome with the longest survival time and performed a literature review 1).


George et al. reported four new cases of primary central nervous system ALCL from the Mayo Clinic and incorporated additional data from five previously published cases. ALK-1 expression was determined in all nine tumors. Patient age was 4-66 years (mean 29 years) with a bimodal distribution: 6 < or = 22 years, 3 > or = 50 years. Six were female. Tumors were mostly supratentorial, five were multifocal, and seven had involvement of dura or leptomeninges. Seven tumors were T cells, two were null cells, and five of nine were ALK-1 immunopositive. Total mortality was six of nine. Three patients, 4-18 years of age (mean 13 years), were alive at 4.8-6.1 years postdiagnosis; these tumors were all ALK-positive. Five patients, 13-66 years of age (mean 43 years), died of tumor 4 days to 11 weeks postdiagnosis; four of five of these tumors were ALK-negative. One 10-year-old child with an ALK-positive tumor died of sepsis, but in remission. The central nervous system ALCL is aggressive. The study suggests that a better outcome may be associated with young age and ALK-1 positivity, prognostic parameters similar to systemic ALCL 2).


A review demonstrated that ALK-negative ALCL exhibits a poor prognosis and is very often fatal. The majority of ALK-negative patients were treated with radiotherapy or supportive care, due to their older age or poor PS. As ALK-negative ALCL tends to occur in older individuals, similar to PCNSL and DLBCL, chemoradiotherapy including HD-MTX should be initiated earlier.

In conclusion, findings indicate that the prognosis of ALCL of the CNS is correlated with ALK positivity and patient age of <40 years. Chemoradiotherapy with MTX is recommended as the standard treatment for ALCL. However, additional multicenter studies including large numbers of cases are required 3).

A 19-year-old male patient was admitted to the hospital complaining of dizzinessCT and MRI imaging showed a heterogeneous enhanced lesion in the left parieto-occipital lobe and the leptomeninges of the occipital lobe and the cerebellum. The lesion was resected and confirmed to be ALK-negative ALCL by pathological examination. Then, the patient received 10 cycles of chemotherapy with high-dose methotrexate (HD-MTX) and whole brain radiotherapy. The patient recovered well and was regularly followed up. He was free of symptoms without recurrence on imaging examination 3 years later. ALCL is a rare type of PCNSL. HD-MTX combined with radiation is an effective therapeutic approach. However, further prospective studies with a large number of patients are needed to identify the biological characteristics of this rare type of PCNSL 4).


1) , 4)

Yuan C, Duan H, Wang Y, Zhang J, Ou J, Wang W, Zhang M. Primary central nervous system ALK-negative anaplastic large cell lymphoma: a case report and literature review. Ann Palliat Med. 2021 Jul 1:apm-21-557. doi: 10.21037/apm-21-557. Epub ahead of print. PMID: 34263607.
2)

George DH, Scheithauer BW, Aker FV, Kurtin PJ, Burger PC, Cameselle-Teijeiro J, McLendon RE, Parisi JE, Paulus W, Roggendorf W, Sotelo C. Primary anaplastic large cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression. Am J Surg Pathol. 2003 Apr;27(4):487-93. doi: 10.1097/00000478-200304000-00008. PMID: 12657933.
3)

Nomura M, Narita Y, Miyakita Y, Ohno M, Fukushima S, Maruyama T, Muragaki Y, Shibui S. Clinical presentation of anaplastic large-cell lymphoma in the central nervous system. Mol Clin Oncol. 2013 Jul;1(4):655-660. doi: 10.3892/mco.2013.110. Epub 2013 Apr 30. PMID: 24649224; PMCID: PMC3915681.

Subdural Evacuating Port System (SEPS)

Subdural Evacuating Port System (SEPS)

http://www.medtronic.com/us-en/healthcare-professionals/products/neurological/critical-care/seps-subdural-evacuation-port-system.html

see Integra™ Subdural Evacuation System.


Twist drill craniostomy (TDC) with closed system drainage and Subdural Evacuating Port System is an effective treatment option for chronic subdural hematoma (CSDH).

In a radiological study, all the branches of the middle meningeal artery ran posterior to the coronal suture and the vascular grooves were also located posterior to the coronal suture at the level of the superior temporal line (STL). The average distance of the vascular grooves was 8.0 +/-5.8 mm. Thirty-five procedures were performed. The coronal suture and the STL could be identified clearly on brain CT scans. The mean thickness of the skull and the CSDH at the proposed point was 8 mm (range 5-13 mm) and 20 mm (range 10-28 mm), respectively. All the TDCs except 1 were congruent with the preoperative brain CT scans. One CSDH recurred 1 month after the first operation and was revised using the same procedure. No other complications occurred.

One centimeter anterior to the coronal suture at the level of the STL is suitable as the normal entry point of the TDC for symptomatic CSDH. The thickness of the CSDH can be measured at this point on a preoperative brain CT scan. Furthermore, the entry point on the scalp can be accurately estimated using surface landmarks 1).


The insertion of a subdural drain was associated with a statistically significant reduction in the risk of symptomatic recurrence and the requirement for further surgical intervention of chronic subdural hematoma after surgical evacuation. Furthermore, it was associated with statistically significant improvements in both short-term and long-term functional outcome 2).

The Subdural Evacuating Port System (SEPS) is a subdural drain that permits the neurosurgeon to drain subacute or chronic subdural hematoma by a method which is minimally invasive, simple and safe to the standard procedure of burr-hole evacuation 3) 4) 5) 6).

The appearance of the winged canula positioned with its tip in the diploic space overlying the subdural space should allow the radiologist to identify it correctly 7).

Because chronic SDH frequently occurs in elderly patients with multiple comorbidities, the bedside approach afforded by the subdural evacuating port system (SEPS) is an attractive alternative method that is performed under local anesthesia and conscious sedation.

A prospectively maintained database of 23 chronic SDHs treated by bur hole or craniotomy and of 23 chronic SDHs treated by SEPS drainage at Tufts Medical Center was compiled, and a retrospective chart review was performed. Information regarding demographics, comorbidities, presenting symptoms, and outcome was collected. The volume of SDH before and after treatment was semiautomatically measured using imaging software.

There was no significant difference in initial SDH volume (94.5 cm(3) vs 112.6 cm(3), respectively; p = 0.25) or final SDH volume (31.9 cm(3) vs 28.2 cm(3), respectively; p = 0.65) between SEPS drainage and traditional methods. In addition, there was no difference in mortality (4.3% vs 9.1%, respectively; p = 0.61), length of stay (11 days vs 9.1 days, respectively; p = 0.48), or stability of subdural evacuation (94.1% vs 83.3%, respectively; p = 0.60) for the SEPS and traditional groups at an average follow-up of 12 and 15 weeks, respectively. Only 2 of 23 SDHs treated by SEPS required further treatment by bur hole or craniotomy due to inadequate evacuation of subdural blood.

This results means thats a safe and effective alternative to traditional methods of evacuation of chronic SDHs and should be considered in patients presenting with a symptomatic chronic SDH 8).

The SEPS is relatively simple to use and may be especially useful to emergency department staff in outlying areas where there is a shortage of neurosurgical coverage 9).

This technique should be added to the armament of treatment options for a neurosurgeon to treat or temporize a hyperacute SDH with increased intracranial pressure in specific patients 10).

Despite decreasing length of stay LOSs as treatment for cSDH evolved from burr holes BHs to SEPS, the LOS for a cSDH is still longer than that of a patient undergoing craniotomy for brain tumor 11).

The efficacy and safety of SEPS is similar to that of other twist-drill methods reported in the literature. The efficacy of this treatment as measured by radiographic worsening or the need for a subsequent procedure is statistically similar to that of bur hole treatment. There was no difference in mortality or other adverse outcomes associated with SEPS 12).

Specifically, hypodense subdural collections drain more effectively through an SEPS than do mixed density collections. Although significant bleeding after SEPS insertion was uncommon, 1 patient required urgent surgical hematoma evacuation due to iatrogenic injury 13).

The SEPS a first-line treatment for the majority of patients with cSDH, management of cSDH must be tailored to each patient. In mixed density collections with large proportions of acute hemorrhage and in collections with numerous intrahematomal septations, alternative surgical techniques should be considered as first-line therapies 14).

Carpenter et al. evaluated the experience with middle meningeal artery embolization (MMA) combined with Subdural Evacuating Port System (SEPS) placement as a first-line treatment for patients with chronic subdural hematoma (cSDH). A single-institution retrospective review was performed of all patients undergoing intervention. Patients were stratified by treatment with MMA embolization and SEPS placement, MMA embolization, and surgery, SEPS placement only, and surgery only for cSDH from 2017 to 2020, and cohorts were compared against each other. Patients treated with MMA/SEPS were more likely to be older, be on anticoagulation, have significant comorbidities, have a shorter length of stay, and less likely to have symptomatic recurrence compared to SEPS only cohort. Thus, MMA/SEPS appears to be a safe and equally effective minimally invasive treatment for chronic subdural hematoma patients with significant comorbidities who are poor surgical candidates 15).


1)

Hwang SC, Im SB, Kim BT, Shin WH. Safe entry point for twist-drill craniostomy of a chronic subdural hematoma. J Neurosurg. 2009 Jun;110(6):1265-70. doi: 10.3171/2008.9.JNS08359. PubMed PMID: 19099378.
2)

Alcalá-Cerra G, Young AM, Moscote-Salazar LR, Paternina-Caicedo A. Efficacy and safety of subdural drains after burr-hole evacuation of chronic subdural hematomas: systematic review and meta-analysis of randomized controlled trials. World Neurosurg. 2014 Dec;82(6):1148-57. doi: 10.1016/j.wneu.2014.08.012. Epub 2014 Aug 10. Review. PubMed PMID: 25118059.
3)

Asfora WT, Schwebach L, Louw D. A modified technique to treat subdural hematomas: the subdural evacuating port system. S D J Med. 2001 Dec;54(12):495-8. PubMed PMID: 11775490.
4)

Asfora WT, Schwebach L. A modified technique to treat chronic and subacute subdural hematoma: technical note. Surg Neurol. 2003 Apr;59(4):329-32; discussion 332. PubMed PMID: 12748020.
5)

Scotton WJ, Kolias AG, Ban VS, Crick SJ, Sinha R, Gardner A, Massey K, Minett T, Santarius T, Hutchinson PJ. Community consultation in emergency neurosurgical research: lessons from a proposed trial for patients with chronic subdural haematomas. Br J Neurosurg. 2013 Oct;27(5):590-4. doi:10.3109/02688697.2013.793291. Epub 2013 Jun 14. PubMed PMID: 23767683.
6)

Singla A, Jacobsen WP, Yusupov IR, Carter DA. Subdural evacuating port system (SEPS)–minimally invasive approach to the management of chronic/subacute subdural hematomas. Clin Neurol Neurosurg. 2013 Apr;115(4):425-31. doi: 10.1016/j.clineuro.2012.06.005. Epub 2012 Jul 3. PubMed PMID: 22763191.
7)

Lollis SS, Wolak ML, Mamourian AC. Imaging characteristics of the subdural evacuating port system, a new bedside therapy for subacute/chronic subdural hematoma. AJNR Am J Neuroradiol. 2006 Jan;27(1):74-5. PubMed PMID: 16418360.
8)

Safain M, Roguski M, Antoniou A, Schirmer CM, Malek AM, Riesenburger R. A single center’s experience with the bedside subdural evacuating port system: a useful alternative to traditional methods for chronic subdural hematoma evacuation. J Neurosurg. 2013 Mar;118(3):694-700. doi: 10.3171/2012.11.JNS12689. Epub 2012 Dec 21. Erratum in: J Neurosurg. 2013 Jul;119(1):256. Schirmer, Clemens S [corrected to Schirmer, Clemens M]. PubMed PMID: 23259822.
9)

Asfora WT, Klapper HB. Case report: treatment of subdural hematoma in the emergency department utilizing the subdural evacuating port system. S D Med. 2013 Aug;66(8):319-21. PubMed PMID: 24175497.
10)

Ivan ME, Nathan JK, Manley GT, Huang MC. Placement of a subdural evacuating port system for management of iatrogenic hyperacute subdural hemorrhage following intracranial monitor placement. J Clin Neurosci. 2013 Dec;20(12):1767-70. doi: 10.1016/j.jocn.2013.03.009. Epub 2013 Oct 3. PubMed PMID: 24090520.
11)

Balser D, Rodgers SD, Johnson B, Shi C, Tabak E, Samadani U. Evolving management of symptomatic chronic subdural hematoma: experience of a single institution and review of the literature. Neurol Res. 2013 Apr;35(3):233-42. doi: 10.1179/1743132813Y.0000000166. Review. PubMed PMID: 23485050.
12)

Rughani AI, Lin C, Dumont TM, Penar PL, Horgan MA, Tranmer BI. A case-comparison study of the subdural evacuating port system in treating chronic subdural hematomas. J Neurosurg. 2010 Sep;113(3):609-14. doi: 10.3171/2009.11.JNS091244. PubMed PMID: 20001585.
13)

Kenning TJ, Dalfino JC, German JW, Drazin D, Adamo MA. Analysis of the subdural evacuating port system for the treatment of subacute and chronic subdural hematomas. J Neurosurg. 2010 Nov;113(5):1004-10. doi: 10.3171/2010.5.JNS1083. Epub 2010 May 28. PubMed PMID: 20509728.
14)

Neal MT, Hsu W, Urban JE, Angelo NM, Sweasey TA, Branch CL Jr. The subdural evacuation port system: outcomes from a single institution experience and predictors of success. Clin Neurol Neurosurg. 2013 Jun;115(6):658-64. doi: 10.1016/j.clineuro.2012.07.017. Epub 2012 Aug 3. PubMed PMID: 22863544.
15)

Carpenter A, Rock M, Dowlati E, Miller C, Mai JC, Liu AH, Armonda RA, Felbaum DR. Middle meningeal artery embolization with subdural evacuating port system for primary management of chronic subdural hematomas. Neurosurg Rev. 2021 Apr 24. doi: 10.1007/s10143-021-01553-x. Epub ahead of print. PMID: 33893872.

Primary Central Nervous System Angiosarcoma

Primary Central Nervous System Angiosarcoma

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to rapid neurological deterioration and death in few months.

Mena et al. reported in 1991 eight patients with primary angiosarcoma of the central nervous system these included five males and three females ranging in age from 2 weeks to 72 years (mean 38 years). Of the eight neoplasms, six were located in the cerebral hemispheres and one was in the meninges; the site was unknown in the other. All patients underwent surgical resection. Five of the eight patients died, four within 4 months after surgery and one after 30 months. Two of the remaining three patients were 17 and 27 years old at the time of diagnosis and were alive at follow-up review 39 and 102 months after surgery, respectively. One patient was lost to follow-up monitoring. Microscopically, all eight tumors demonstrated a well-differentiated pattern with irregular vascular channels and intraluminal papillae; in addition, four showed poorly differentiated solid areas. Immunohistochemical staining of neoplastic cells to factor VIII-related antigen and Ulex europaeus agglutinin I was performed in five tumors and was focally positive in four. No correlation could be shown between the histological features and the growth and biological behavior of the tumors 1)

Valera-Melé et al. presented a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the dura mater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice 2).


Gao M, Li P, Tan C, Liu J, Tie X, Pang C, Guo Z, Lin Y. Primary Central Nervous System Angiosarcoma. World Neurosurg. 2019 Dec;132:41-46. doi: 10.1016/j.wneu.2019.08.128. Epub 2019 Aug 27. PubMed PMID: 31470162 3).


report a case of intracranial angiosarcoma in a Caucasian male and present a review of the imaging features in the recent literature. The tumor mostly presents as a well-demarcated, heterogeneous, moderately to strongly enhancing lesion with signs of intratumoral bleeding and surrounding vasogenic edema. The differential imaging features of common hemorrhagic intracranial tumors are discussed 4).


Two cases of primary angiosarcoma of the brain are well characterized by imaging, histopathology, and immunohistochemistry. Case 1: The first patient was a 35-year-old woman who developed exophthalmos. Subtotal resection of a left extra-axial retro-orbital mass was performed.

Case 2: our second patient was a 47-year-old man who presented with acute visual loss, word-finding difficulty, and subtle memory loss. A heterogeneously-enhancing left sphenoid wing mass was removed. We also review the literature aiming at developing a rational approach to diagnosis and treatment, given the rarity of this entity.

Gross total resection is the standard of care for primary angiosarcoma of the brain. Adjuvant radiation and chemotherapy are playing increasingly recognized roles in the therapy of these rare tumors 5).


1)

Mena H, Ribas JL, Enzinger FM, Parisi JE. Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature. J Neurosurg. 1991 Jul;75(1):73-6. doi: 10.3171/jns.1991.75.1.0073. PMID: 2045922.
2)

Valera-Melé M, Darriba Allés JV, Ruiz Juretschke F, Sola Vendrell E, Hernández Poveda JM, Montalvo Afonso A, Casitas Hernando V, García Leal R. Primary central nervous system angiosarcoma with recurrent acute subdural hematoma. Neurocirugia (Astur). 2021 Mar 22:S1130-1473(21)00027-0. English, Spanish. doi: 10.1016/j.neucir.2021.02.002. Epub ahead of print. PMID: 33766476.
3)

Gao M, Li P, Tan C, Liu J, Tie X, Pang C, Guo Z, Lin Y. Primary Central Nervous System Angiosarcoma. World Neurosurg. 2019 Dec;132:41-46. doi: 10.1016/j.wneu.2019.08.128. Epub 2019 Aug 27. PubMed PMID: 31470162.
4)

Jerjir N, Lambert J, Vanwalleghem L, Casselman J. Primary Angiosarcoma of the Central Nervous System: Case Report and Review of the Imaging Features. J Belg Soc Radiol. 2016 Oct 10;100(1):82. doi: 10.5334/jbr-btr.1087. PMID: 30151480; PMCID: PMC6100495.
5)

Hackney JR, Palmer CA, Riley KO, Cure JK, Fathallah-Shaykh HM, Nabors LB. Primary central nervous system angiosarcoma: two case reports. J Med Case Rep. 2012 Aug 21;6:251. doi: 10.1186/1752-1947-6-251. PMID: 22909122; PMCID: PMC3459733.
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