Clinical spectrum of a syndromic diagnosis: Lethal midline granuloma (original) (raw)

Extranodal Natural Killer/T-cell lymphoma, nasal type: ‘midline lethal granuloma.’ A case report

Head & Face Medicine, 2013

Extranodal natural killer/T cell lymphoma, nasal type, is a non-Hodgkin lymphoma, most commonly affecting the nasal cavity, paranasal sinuses and nasopharynx. Clinically it is characterised by destruction of facial tissues, commencing in the midline. In most cases it arises from malignant transformation of natural killer cells (NK); sometimes from malignant transformation of cytotoxic T cells. Extranodal NK/T cell lymphoma, nasal type, is rare, but even more rare in black persons. The purpose of this article is to report a severe case of extranodal NK/T cell lymphoma, nasal type, in an elderly black male.

Nasal-type NK/T-cell lymphoma: a case report

Acta dermatovenerologica Alpina, Pannonica, et Adriatica, 2007

Extranodal NK/T-cell lymphoma represents less than 1% of all lymphomas, but is more common in Asia and South America. We present a 67-year-old female with a 10-month history of four reddish-blue firm and painful nodules in the parietal region of the head, ranging in size from 1 to 5 cm. Two nodules were taken for biopsy, which showed atypical lymphoid cells with angiocentric growth pattern. The immunophenotype of the tumor cells was CD45RO +, CD56 +, CD3 + (epsilon chain), CD20-, consistent with the diagnosis of NK/T-cell lymphoma. NK/T-cell lymphomas are rare and the optimal treatment has not been clearly established.

Extranodal NK/T-Cell Lymphoma, Nasal Type—Case Report of 2 Cases

Ear, nose, & throat journal, 2019

A 17-year-old male was admitted to hospital for fever, right periorbital edema, purulent rhinorrhea, and nasal obstruction for 3 days (Figure 1A). He had been under treatment with antibiotic and corticoid for rhinosinusitis, with no improvement. Previous surgery was performed 6 months before for ''nasal polyposis.'' The anatomopathological (AP) highlighted polypoid inflammatory mucosa. Upon examination, he presented with peripalpebral edema on the right, conjunctival hyperemia with preserved ocular mobility and visual acuity and painless subcutaneous nodules on the right hemiface. Nasoendoscopy showed no changes in the left or nasopharynx; in the right, edema and mucosa hyperemia obstructing the upper airway and purulent rhinorrhea. Contrast computerized tomography (CT) of sinuses and orbit revealed extraconal infiltrating and expanding injury compromising orbit and right periorbit, with eyeball proptosis; maxillary, sphenoid sinus, ethmoidal cells, frontal sinus, and nasolacrimal duct were obliterated; and infiltration of the skin and subcutaneous on the right was identified (Figure 1B and C). Abdomen CT revealed splenomegaly (15.5 cm). Pelvis and chest CT were normal. Serologies, rheumatic tests, hemoculture, uroculture, and Mantoux were negative. During admission, cefepime was commenced for febrile neutropenia. Biopsy of the lesion via nasal endoscopy in the right nasal cavity was performed with Epstein-Barr virus (EBV) checking positive. There was not enough material for diagnosis through immunohistochemistry due to the large amount of necrotic material. There was a gradual worsening of pancytopenia, increase in lactate dehydronagenase (LDH), and episode of right severe epistaxis, which was controlled with nasal packing. Bone marrow biopsy was carried out due to the suspicion of lymphoma, with negative result. Prednisone was initiated in lower doses, and new biopsy of the lesion in the right nasal cavity was performed through lateral rhinotomy, anatomopathology examination identified lymphoma. Extranodal natural killer /T-cell lymphoma, nasal type (ENKL) was confirmed by immunohistochemistry. Treatment with radiotherapy and outpatient chemotherapy was commenced.

2016 An B Derm _ P de Vasconcelos _ Multifocal PCEN NK_T lymphoma nasal type.pdf

Nasal type extranodal NK/T-cell lymphoma is a distinct entity according to the World Health Organization classification. Although 60% to 90% of patients with this disease present with a destructive mass in the midline facial tissues, it may also primarily or secondarily involve extranasal sites, like the skin. We report the case of a 77-year-old patient that came to our department with erythematous plaques of the right leg and eczematous lesions of the trunk. These lesions were biopsied and the patient was diagnosed with extranodal NK/T-cell lymphoma, nasal type. He was treated with multi-agent systemic chemotherapy but died 5 months after diagnosis. This case highlights the rarity and variability of cutaneous features of this disease and its aggressive course and poor prognosis.

NK cell lymphoma, nasal type, with massive lung involvement: a case report

Journal of Hematopathology, 2010

Extranodal NK/T cell lymphoma, nasal type, is an Epstein-Barr virus-associated lymphoma that most commonly involves the nasal cavity and upper respiratory tract. Lung involvement by NK/T cell lymphoma is rare and seldom reported in the literature. We describe the unusual case of a 41-year-old male with NK cell lymphoma, nasal type, who presented with massive secondary lung involvement 2.5 years after the detection of a retroperitoneal mass.