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neural tendon sheath fibromas, giant cell flexor sheath tumors, and intraneural lipomas [1–5]. Extraneural tendon sheath fibromas are an unrelated myeloproliferative process. In this patient, the mass arose intraneurally and developed rapidly with resulting median neuropathy. To the best of out knowledge, this is the first reported case of a histologically confirmed intraneural fibroma. Given these findings, intraneural fibromas should be included in the differential diagnosis of median neuropathy with a mobile wrist mass. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
References [1] Okubo T, Saito T, Mitomi H, et al. Intraneural lipomatous tumor of the median nerve: three case reports with a review of literature. Int J Surg Case Rep 2012;3: 407–11. [2] Tiong WH, Ismael TS, Regan PJ. Fibroma of tendon sheath: a rare cause of carpal tunnel syndrome. J Hand Surg 2006;31:579–80. [3] Chalmers RL, Mandalia M, Contreras R, et al. Acute trigger wrist and carpal tunnel syndrome due to giant-cell tumour of the flexor sheath. J Plast Reconstr Aesthet Surg 2008;61:1557. [4] Evangelisti S, Reale VF. Fibroma of tendon sheath as a cause of carpal tunnel syndrome. J Hand Surg 1992;17:1026–7. [5] Garrido A, Lam WL, Stanley PR. Fibroma of a tendon sheath at the wrist: a rare cause of compression of the median nerve. Scand J Plast Reconstr Surg Hand Surg 2004;38:314–6.
http://dx.doi.org/10.1016/j.jocn.2013.10.002
Primary leptomeningeal melanocytosis presenting as chronic meningitis Michael C. Honigberg c,⇑, Efstathios Papavassiliou b,c, Yehuda Z. Cohen a,c a
Division of Infectious Diseases, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA Division of Neurosurgery, Department of Surgery, Beth Israel Deaconess Medical Center, Boston, MA, USA c Tosteson Medical Education Center, Harvard Medical School, 260 Longwood Avenue, Boston, MA 02115, USA b
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Article history: Received 17 October 2013 Accepted 18 October 2013
Keywords: CSF protein elevation Leptomeningeal melanocytic neoplasm Meningeal melanocytosis Primary leptomeningeal melanocytosis
a b s t r a c t We report a patient with primary leptomeningeal melanocytosis presenting as chronic meningitis. A previously healthy 27-year-old man presented with 2 months of severe headaches and photophobia. A lumbar puncture was notable for a highly elevated cerebrospinal fluid (CSF) protein level without pleocytosis. Imaging at the time of admission suggested only meningitis without the presence of parenchymal lesions. On the basis of the CSF findings, early meningeal biopsy was performed, leading to the diagnosis of a meningeal melanocytic neoplasm. Early meningeal biopsy should be considered in patients with meningitis when the CSF profile suggests the possibility of a central nervous system neoplasm. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Primary leptomeningeal melanocytosis is a rare central nervous system (CNS) neoplasm with an estimated incidence of 1 in 10 million [1]. It can have a variety of clinical presentations, including headache, neck pain, symptoms of increased intracranial pressure, focal neurologic deficits, and seizures [2,3]. We report a previously healthy young man who presented with chronic meningitis and was ultimately found to have primary leptomeningeal melanocytosis.
2. Case report A 27-year-old man originally from El Salvador was transferred to our hospital with confusion and 2 months of headache. Seven weeks previously, he had presented to an outside hospital with 2 weeks of severe headache as well as nausea, vomiting, and mild photophobia. A lumbar puncture (LP) was performed and the results are shown in Table 1. An infectious workup was negative. The patient was discharged with presumed aseptic meningitis. He continued to have daily headaches with nausea and vomiting. One week prior to admission, he presented to the same outside hospital and underwent a second LP (Table 1). A tuberculin skin ⇑ Corresponding author. Tel.: +1 703 980 4521; fax: +1 617 735 4527. E-mail address:
[email protected] (M.C. Honigberg).
test was positive and cerebrospinal fluid (CSF) mycobacterial culture was sent. He was discharged without a clear diagnosis. Six days later, the patient was transferred to our institution with confusion and lethargy. MRI of the head with contrast showed diffuse leptomeningeal enhancement without parenchymal lesions (Fig. 1A, B). A third LP was performed, and the patient was scheduled for meningeal biopsy (Table 1). Intra-operatively, multiple black and gray spots were visualized on the dura (Fig. 2). Pathology revealed a melanocytic neoplasm in the subarachnoid and Virchow-Robin spaces with significant atypia and a high mitotic index, consistent with a primary CNS melanocytic meningeal tumor or metastatic melanoma. A lesion on the patient’s right leg was concerning for melanoma; however, biopsy revealed a benign nevus. The patient was diagnosed with primary leptomeningeal melanocytosis. He received whole-brain radiation without neurologic improvement. Repeat head MRI on hospital day 45 demonstrated progression of disease (Fig. 1C, D). The patient died on hospital day 53.
3. Discussion In this young man from El Salvador with chronic meningitis, a broad differential diagnosis was considered, including infectious etiologies such as tuberculosis (TB) meningitis. However, based on the CSF findings, early meningeal biopsy was performed, establishing the diagnosis of leptomeningeal melanocytosis.
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Case Reports / Journal of Clinical Neuroscience 21 (2014) 1056–1058 Table 1 Results of cerebrospinal fluid examination and opening pressure on serial lumbar punctures
RBC (cells per lL) WBC (cells per lL) WBC differential: Neutrophils Lymphocytes Monocytes Macrophages Other Protein (mg/dL) Glucose (mg/dL) Opening pressure (cm H2O)
Seven weeks prior to admission
One week prior to admission
On admission
3 0
58 18
15 3
N/A
3% 50% 39% 0% 8% ‘‘scattered atypical mononuclear cells, likely reactive’’
10% 16% 15% 52% 7% ‘‘reactive and plasmacytoid lymphocytes’’
137 37 26
300 58 36
870 111 >60
RBC = red blood cell, WBC = white blood cell.
Fig. 2. Intra-operative photograph showing craniotomy with exposed dura. Black spots, representing areas of melanocytosis, are visualized overlying the dura (arrows). (This figure is available in colour at www.sciencedirect.com)
Fig. 1. Post-contrast T1-weighted axial MRI at the level of the midbrain (A, C) and lateral ventricles (B, D). Imaging obtained at presentation (A, B) shows leptomeningeal enhancement, consistent with meningitis. Repeat imaging (C, D) shows disease progression with increased thickness of leptomeningeal enhancement and invasive spread into the mesial temporal lobes (C).
One of the most striking features of this case was the patient’s extremely elevated CSF protein level of 870 mg/dL (normal range 15–45 mg/dL). TB meningitis and fungal CNS infections only rarely cause such marked CSF protein elevation [4–7]. Amebic CNS infection may occasionally elevate CSF protein above 1000 mg/dL [8]. Rheumatologic conditions such as neuro-sarcoid, neuro-lupus, and neuro-Behçet’s may also cause marked CSF protein elevation [9]. Neoplastic meningitis may result in very high CSF protein levels as well [10]. The other notable feature of our patient’s CSF profile was its lack of cellularity. While hypocellular CSF is extremely rare in infectious
and inflammatory meningitides, it is relatively common in neoplastic involvement of the meninges. In one series of 63 patients with leptomeningeal metastases diagnosed by CSF cytology, 36% of patients had CSF with less than four white blood cells/lL [11]. In another similar series of 45 patients, only 33% had CSF pleocytosis, although 76% had elevated CSF protein [12]. Most cases of leptomeningeal melanocytosis appear to present in children who also have large dysplastic nevi, constituting a syndrome known as neurocutaneous melanosis [13]. Leptomeningeal melanocytosis may have a wide range of clinical presentations, and presentation depends on the distribution of melanocytosis, rate of tumor growth, and presence or absence of parenchymal invasion [2,3]. Symptomatic leptomeningeal melanocytosis carries an extremely poor prognosis, and palliative treatment is often pursued. Chemotherapy has not been shown to prevent progression or death [1]. We report a patient who presented with chronic meningitis caused by leptomeningeal melanocytosis, a rare CNS neoplasm and even rarer cause of chronic meningitis. Imaging at the time of admission suggested only meningitis without the presence of parenchymal lesions, but based on the CSF findings of a highly elevated protein level in combination with hypocellularity, early meningeal biopsy was performed, leading to the diagnosis. Close attention to CSF findings can lead to early biopsy and more rapid diagnoses in challenging cases such as this one.
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Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Liubinas SV, Maartens N, Drummond KJ. Primary melanocytic neoplasms of the central nervous system. J Clin Neurosci 2010;17:1227–32. [2] Ramaswamy V, Delaney H, Haque S, et al. Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol 2012;54:563–8. [3] Arsene D, Ardeleanu C, Balescu C, et al. Meningeal melanocytosis in a young patient–an autopsy diagnosis. Clin Neuropathol 2007;26:294–8. [4] Garg RK. Tuberculosis of the central nervous system. Postgrad Med J 1999;75:133–40. [5] Thompson 3rd GR, LaValle 3rd CE, Everett ED. Unusual manifestations of histoplasmosis. Diagn Microbiol Infect Dis 2004;50:33–41. [6] Mathison G, Shelub A, Truong J, et al. Coccidioidal meningitis: clinical presentation and management in the fluconazole era. Medicine (Baltimore) 2010;89:251–84.
[7] Bariola JR, Perry P, Pappas PG, et al. Blastomycosis of the central nervous system: a multicenter review of diagnosis and treatment in the modern era. Clin Infect Dis 2010;50:797–804. [8] Visvesvara GS, Moura H, Schuster FL. Pathogenic and opportunistic free-living amoebae: Acanthamoeba spp., Balamuthia mandrillaris, Naegleria fowleri, and Sappinia diploidea. FEMS Immunol Med Microbiol 2007;50:1–26. [9] Reske D, Petereit HF, Heiss WD. Difficulties in the differentiation of chronic inflammatory diseases of the central nervous system – value of cerebrospinal fluid analysis and immunological abnormalities in the diagnosis. Acta Neurol Scand 2005;112:207–13. [10] Chamberlain MC. Leptomeningeal metastasis. Curr Opin Oncol 2010;22:627–35. [11] Kaplan JG, DeSouza TG, Farkash A, et al. Leptomeningeal metastases: comparison of clinical features and laboratory data of solid tumors, lymphomas and leukemias. J Neurooncol 1990;9:225–9. [12] van Oostenbrugge RJ, Twijnstra A. Presenting features and value of diagnostic procedures in leptomeningeal metastases. Neurology 1999;53:382–5. [13] Sutton BJ, Tatter SB, Stanton CA, et al. Leptomeningeal melanocytosis in an adult male without large congenital nevi: a rare and atypical case of neurocutaneous melanosis. Clin Neuropathol 2011;30:178–82.
http://dx.doi.org/10.1016/j.jocn.2013.10.014
Salivoma after carotid endarterectomy Asif Bashir a,1, Mohammed Hussain a,1, Haitham Dababneh a, Deborah Rosin b, Aaron A. Cohen-Gadol c,⇑ a
New Jersey Neuroscience Institute, Department of Neurosurgery, JFK Medical Center, Seton Hall University, Edison, NJ, USA Department of ENT, JFK Medical Center, Edison, NJ, USA c Goodman-Campbell Brain and Spine, Department of Neurological Surgery, Indiana University School of Medicine, 355 W. 16th Street, Suite 5100, Indianapolis, IN 46202, USA b
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Article history: Received 18 September 2013 Accepted 19 October 2013
Keywords: Complications Endarterectomy Salivary glands Salivoma
a b s t r a c t Although the diagnosis and management of postoperative or traumatic fluid collections have been documented extensively in the literature, to our knowledge the occurrence of a salivoma after carotid endarterectomy has not been reported. We report an extra salivary glandular collection of saliva – a ‘‘salivoma’’ – in a 79-year-old patient who underwent a carotid endarterectomy with a high carotid bifurcation. He presented with serous watery drainage from the incision site that had started spontaneously 4 days after surgery. The patient was taken to the operating room for exploration and washout of the wound with presumption of an infectious source. As self-retaining retractors were placed under the platysma, a large release of serous fluid occurred. Copious irrigation allowed complete washout of the wound. On postoperative day 2, the patient re-exhibited neck wound fullness and a Penrose drain was placed in the incision with clear serous fluid flowing through the drain. The patient was given a scopolamine patch to decrease salivary secretions. Within 5 days, the drainage significantly decreased and the drain was removed. This diagnosis should be included in the differential diagnosis of an expanding neck mass following carotid endarterectomy to properly treat this complication. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction The literature abounds with studies on the formation and treatment of postoperative fluid collections such as hematomas and seromas. They usually arise from insufficient wound healing or mechanical failure at the surgical site [1]. The otolaryngology literature also describes formation of ‘‘sialoceles’’ or subcutaneous cavities composed of saliva, usually resulting from trauma or infection of the parotid gland parenchyma, laceration of the parotid duct, or ductal stenosis with subsequent dilatation [2]. Characterized as swelling around the parotid region, these lesions are soft and mobile with patients experiencing no pain, fever, chills, or erythema unless secondarily infected [3]. These sialoceles usually result from trauma such as sharp penetrating wounds in the oral cavity or face or may be secondary to blunt trauma, such as zygomatic and man⇑ Corresponding author. Tel.: +1 317 362 8760; fax: +1 317 924 8472. 1
E-mail address:
[email protected] (A.A. Cohen-Gadol). These authors have contributed equally to the manuscript.
dible fractures [4,5]. The history usually includes facial trauma or surgery days or weeks before the onset of swelling [2]. Extra-salivary pocket formations of saliva in patients who have undergone vascular procedures have seldom been reported. We report a patient after carotid endarterectomy with a high carotid bifurcation who developed a well-demarcated extra salivary glandular pocket of saliva or ‘‘salivoma’’. Diagnosis of salivoma after carotid endarterectomy is complex and requires clinical suspicion and a thorough history and assessment. Proper management involves initiating prophylactic antibiotics along with adequate drainage to prevent wound dehiscence and infection.
2. Case report A 79-year-old right-handed man with a history of coronary stents on Plavix (Bristol-Myers Squibb, New York, NY, USA) presented with acute onset of mild left hemiparesis and hemisensory loss. Brain MRI showed small multifocal cortical infarcts in the
