T-Cell Acute Lymphoblastic Lymphoma Presenting as Dissecting Cellulitis and Cavernous Sinus Syndrome

Clinical Correspondence T-Cell Acute Lymphoblastic Lymphoma Presenting as Dissecting Cellulitis and Cavernous Sinus Syndrome Jeffrey J. Mattingly, MD, Ama Sadaka, MD, Andrew G. Lee, MD, Sushma S. Yalamanchili, MD A 29-year-old Hispanic man with no significant medical history presented to a primary care provider with "bumps" on his scalp, consisting of approximately 20 subcutaneous nodules without alopecia (Fig. 1). He was diagnosed with "dissecting cellulitis" of the scalp and prescribed oral sulfamethoxazole and trimethoprim (Bactrim), but his symptoms did not improve. A dermatologist agreed with the clinical diagnosis of "dissecting cellulitis" and prescribed oral clindamycin. The patient experienced moderate symptomatic improvement. Two months later, he developed left-sided ptosis, horizontal diplopia that was worse in right gaze, left periorbital pain and headaches, and left facial allodynia. Examination demonstrated complete ptosis and limitation of adduction and infraduction of the left eye. Evaluation for infectious etiologies was unrevealing, including brain MRI and magnetic resonance angiography that were read as "negative." Eleven days later, the patient presented to the emergency department with worsening headaches. Ophthalmology was consulted, and the patient reported left facial numbness, unintentional weight loss, and severe, migrating pains in his extremities. His physical examination demonstrated scalp nodules and left trigeminal hypoesthesia. Ophthalmic examination showed complete left ptosis and ophthalmoplegia. Review of the initial MRI was suspicious for left cavernous sinus disease. Repeat MRI demonstrated a mass at the skull base involving the left orbital apex, both cavernous sinuses and the sella turcica (Fig. 2). There was also perineural spread along both third nerves. On lumbar puncture, cerebrospinal fluid (CSF) analysis revealed 21 white blood cells with 95% lymphocytes and 5% monocytes. Cytological analysis of the CSF showed an atypical lymphoid population consistent with T-cell lymphoblastic lymphoma (T-LBL). Results of a punch biopsy of the scalp were also consistent with T-LBL, showing a diffuse dermal lymphocytic infiltration with high-grade features. Serum and bone marrow samples were negative for blast cells. Computed tomography of the thorax demonstrated a large, anterior mediastinal mass. Hematology-oncology and radiation oncology initiated focal radiation therapy and cyclophosphamide, vincristine, doxorubicin and dexamethasone with subsequent improvement of his cranial neuropathies, including complete resolution of ptosis. At the time of last followup, he had completed chemotherapy through Cycle 3B, received intrathecal methotrexate and cytarabine, and was awaiting stem-cell transplantation. T-LBL is a rare variant of non-Hodgkins lymphoma, comprising only 2% of cases (1). Concordant with its Department of Ophthalmology (JJM, AS, AGL, SSY), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Department of Ophthalmology and Visual Sciences (JJM, AGL), University of Texas Medical Branch at Galveston, Galveston, Texas; Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medical College, New York, New York; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Address correspondence to Jeffrey J. Mattingly, MD, 700 University Boulevard, Galveston, TX 77550; E-mail: [e-mail] 470 FIG. 1. The scalp shows subcutaneous nodules without overlying alopecia. Mattingly et al: J Neuro-Ophthalmol 2018; 38: 470-472 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 2. Contiguous postcontrast coronal T1 scans from anterior (A) to posterior (F) demonstrate a skull base mass involving both cavernous sinuses (left . right), with extension through the sella and infiltration of both Meckel caves. composition of immature precursor T cells, it has a relatively early age at onset (median age at diagnosis: 20 years) and poor prognosis (5-year survival rate for adults: 26%) (2). The clinical manifestations of T-LBL differ from B-LBL, and T-cell disease differs further among age groups (3). Central nervous system involvement is rare (2%), although more common among T-cell lymphomas compared with Bcell lineages. For T-LBL, mediastinal involvement often occurs, spleen and liver enlargement is somewhat less frequent, and severe leukocytosis is less common over the age of 30 years. Our patient was initially misdiagnosed with dissecting cellulitis of the scalp (DCS). This is a chronic, inflammatory, relapsing disease typically affecting in young, African American men with painful, suppurative, subcutaneous nodules and alopecia due to follicular destruction (4). The cause is believed to be autoimmune with secondary bacterial infection. Our patient's ethnicity, subacute course, and lack of alopecia made DCS less likely. In a review of 13 cases of cutaneous T-LBL, the most common sites of involvement were the head and neck at Mattingly et al: J Neuro-Ophthalmol 2018; 38: 470-472 77%, cumulatively (1). The lesions tended to appear as nodules, especially with infiltration into deeper tissues and structures. Notably, 38% had mediastinal involvement. Reports of T-LBL involving the cavernous sinus are rare. Sadruddin et al (2) documented the case of a 17-year-old with right cavernous sinus invasion leading to deficits of the third, fourth, ophthalmic, and maxillary nerves. Wiens et al (5) described a 58-year-old woman with T-LBL infiltrating the pituitary gland and extending into the right cavernous sinus, causing a right third nerve palsy. The differential diagnosis of bilateral cavernous sinus syndrome is largely synonymous with that of a central skull base mass, comprising pituitary neoplasm, meningioma, lymphoma, and skeletal tumors such as osteosarcoma or bony metastases including prostatic adenocarcinoma, with its predilection for the greater wing of the sphenoid (6). Perineural spread may be seen in inflammatory, infectious, or neoplastic diseases, particularly with adenoid cystic and squamous cell carcinomas. Pathologies with meningeal spread also warrant consideration, including infectious and 471 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence inflammatory etiologies as well as leukemia, melanoma, and solid malignancies of the breast and lung (7). Our case serves to reinforce the etiologic heterogeneity of cavernous sinus syndrome and the importance of identifying systemic features to guide clinical management. Cutaneous signs of T-LBL, or lymphoma more broadly, can serve as the pivotal biopsy site which could yield an earlier diagnosis with improved outcomes. REFERENCES 1. Ginoux E, Julia F, Balme B, Thomas L, Dalle S. T-lymphoblastic lymphoma with cutaneous involvement. World J Clin Cases. 2015;3:727-731. 2. Sadruddin S, Medeiros LJ, DeMonte F. Primary T-cell lymphoblastic lymphoma of the cavernous sinus. J Neurosurg Pediatr. 2010;5:94-97. 472 3. Chiaretti S, Vitale A, Cazzaniga G, Orlando SM, Silvestri D, Fazi P, Valsecchi MG, Elia L, Testi AM, Mancini F, Conter V, te Kronnie G, Ferrara F, Di Raimondo F, Tedeschi A, Fioritoni G, Fabbiano F, Meloni G, Specchia G, Pizzolo G, Mandelli F, Guarini A, Basso G, Biondi A, Foà R. 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