Its management requires skilled physicians in order to suspect this disease and making a proper diagnosis at early stages. This paper reports the case of a year-old male patient, with one month of nasal obstruction, recurrent sinusitis, palatal ulceration and a necrotizing lesion. Histopathology reported lymphoid infiltrate polymorph angiocentric growth pattern and extensive areas of necrosis. IgG for Epstein-Barr virus was also positive.
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A case Report. Department of Otorhinolaryngology — Faculty of Medicine —. Universidad Nacional de Colombia. Liliana Ramos—Valencia, MD. Corresponding author:. Luis Felipe Romero Moreno. Email: lfromerom unal. Calle 83 A- N A- Casa Its management requires skilled physicians in order to suspect this disease and making a proper diagnosis at early stages.
This paper reports the case of a year-old male patient, with one month of nasal obstruction, recurrent sinusitis, palatal ulceration and a necrotizing lesion. Histopathology reported lymphoid infiltrate polymorph angiocentric growth pattern and extensive areas of necrosis. IgG for Epstein-Barr virus was also positive.
This case is a clear example of the importance of early diagnostic through multiple biopsies in order to establish a specific treatment to decrease complication rates and improve prognosis. This lymphoma is one of the most lethal midline granulomas, which are characterized for the extensive destructive lesions in the mucosa of the superior airway and middle facial third 1,2. A correct diagnosis is important to define the type of treatment, which may include surgical procedures, chemotherapy or radiotherapy 8.
He works as a painter, with constant exposure to inhaled chemicals. The month prior to consultation, he was treated for acute sinusitis with amoxicillin for 10 days due to bilateral nasal obstruction and occasional epistaxis. Three weeks before consultation, he noted a painful ulcer in the hard palate, although, no systemic symptoms were reported.
On general examination he was cachectic but stable, and presented with hyponasal speech. Rhinoscopy showed a bilateral mass in the nasal cavity with septal perforation in Cottle zones 2 — 3.
Hard and soft palate showed a necrotic lesion with irregular borders, mucosa swelling without active bleeding. He had no palpable adenopathy on head and neck Figure 1. Figure 1. Ulcerated lesion in hard palate with necrotic borders. Circle: Delimitation of the site of the biopsy. Source: Own elaboration based on the data obtained in the study. Computed tomography scan in face and neck showed a mass occupying the nasal cavity, extended to the nasopharynx.
The paranasal sinuses were occupied by homogeneous soft tissue density material; left lamina papyracea was eroded and the periorbital fat had inflammatory changes Figure 2. Lymph nodes of the neck were compromised at zones IIa y IIb. The upper airway was patent. No lesions were identified in CT scans of thorax and abdomen. The patient was hospitalized and multiple punch biopsies of the soft palate were taken.
Figure 2. CT scan of nose and paranasal sinuses. Coronal view: complete occupation of left maxillary sinus, frontal and bilateral ethmoidal sinuses. Arrow: Eroded left lamina papyracea.
Broad spectrum antibiotic and pain control treatment were administrated, but during the first week of hospitalization the patient presented left proptosis and gradual loss of vision. He was taken to the operation room for a left lateral canthotomy, but three days later, the patient developed irreversible left amaurosis. The histopathology test for the soft palate biopsy reported polymorphous lymphoid infiltrate with angiocentric distribution and extensive necrosis Figure 3.
Figure 3. Soft palate biopsy. Polymorphous lymphoid infiltrate with angiocentric distribution. Only 2 courses of SMILE were administered with adequate tolerance and some side effects, which were easily managed, such as queasiness, vomiting, weight loss and alopecia.
Figure 4 shows the improvement of the lesion seven months after initiation of treatment; finally, after ten months of clinical and radiological surveillance, there was a complete remission of the lesions in oral and nasal cavity. T able 1. SMILE chemotherapy regimen 8. Most of the lethal midline granulomas correspond to NK-cell lymphomas, in which an angiocentric and angiodestructive lymphocytic proliferation occurs along the midline tissue with a fast growing rate.
The initial symptoms are non-specific and include nasal obstruction and rhinorrhea with recurrent bacterial sinusitis 5,6. As the disease progresses, a unilateral collapse of the nasal cavity and oronasal fistula may appear due to edema, necrosis and major destruction of the tissue in the facial midline 6. Cutaneous manifestations have the highest prevalence among systemic symptoms. Figure 4.
Initial lesion in the palate. The most accurate test for diagnosis in order to find atypical lymphocytic infiltrates with angiocentric distribution is biopsy, and immunohistochemistry is always positive for tumoral markers CD3, CD4, CD56, CD40, CD40 RO. Medical imaging with computed tomography and magnetic resonance are useful for determining the size of the lesion, the presence of osteolysis, as well as extension to adjacent structures 8,9.
Early diagnosis is of great relevance for better prognosis in order to achieve a higher survival rate. Bad prognosis markers at the time of diagnosis are extensive local invasion, lymph nodes compromise, metastases, high levels of LDH, history of EVB infection and systemic inflammatory response syndrome 6.
Likewise, classifying the disease based on the TNM system is essential to define the treatment and the prognosis 10 Table 2. Numerous scales of functionality for oncologic patients have been described; therefore, ECOG performance status and NCCN—IPI are recommended since they are the most effective and practical ways to define the stage and prognosis of a lymphoma 11, T able 2. Anterior ethmoidal sinus, maxilar sinus, hard palate. Posterior ethmoidal sinus, sphenoid sinus, frontal sinus, oral cavity.
Alveolar process, infratemporal fossa, intracraneal fossa. The treatment depends on the staging of the disease. Better results have been accomplished with doses higher than 50 Gy Multiple protocols of chemotherapy are described with asparaginase, cyclophosphamide, etoposide, among others, which can be used in advanced stages, but survival rates have a clear decrease Stem cell transplant in advanced stages and relapses is currently being considered as an alternative therapy with good results for improving quality of life and higher survival rates 14, Multidisciplinary approaches of this disease are fundamental for the treatment of the patients; new studies are required to evaluate possible alternatives for mid-face reconstruction in patients without relapse.
Emphasizing on the early diagnosis of one of the most lethal midline pathologies can improve prognosis and quality of life. Although, diagnosis is made based on the first biopsy, usually, more than two or three biopsies are necessary.
For advanced stages, only chemotherapy is mandatory in order to reduce mortality probabilities. Braz J Otorhinolaryngol. Lethal midline granuloma. Indian Dermatol Online J. Acta otorrinolaringol Esp. Head Face Med. Actas Dermosifiliogr. J Mycol Med. Clin Lymphoma Myeloma Leuk. Medicine Baltimore. Nasal T- cell lymphoma and lethal midline granuloma syndrome. Otolaryngol Head Neck Surg. Lethal medline granuloma in a human inmunodeficiency virus-infected patient.
Am J Med. J Clin Oncol. Biol Blood Marrow Transplant ; 14 12 Corresponding author: Luis Felipe Romero Moreno. Confined to nasal cavity. With or without nodular compromise. Local or far metastases.
A case Report. Department of Otorhinolaryngology — Faculty of Medicine —. Universidad Nacional de Colombia. Liliana Ramos—Valencia, MD. Corresponding author:. Luis Felipe Romero Moreno. Email: lfromerom unal.
Granuloma letal de la linea media
Wolff S. En: Harrison Principios de Medicina Interna. Idiopathic midline destructive disease-case report and review of the literature. Postgraduate Medical Journal, ; Association of Epstein-Barr virus letal with letal midline granuloma. NEJM, ; Harabuchi Y, Yamanaka N.
Lethal midline granuloma
Lethal midline granuloma LMG is an historical term for a condition in which necrotic and highly destructive lesions develop progressively in the middle of the face, principally the nose and palate. Many cases presented with ulcerations in or perforations of the palate. LMG was thought to be a manifestation of three  or four  different diseases: the well-characterized disease of granulomatosis with polyangiitis , the ill-defined disorders of polymorphic reticulosis or mid-line malignant reticulosis, and an incompletely defined form of non-Hodgkin's lymphoma. Subsequent studies found that the cells infiltrating the midline tissues in cases of lethal midline granuloma that were not clearly diagnosed as granulomatosis with polyangiitis were: a infected by the Epstein-Barr virus  and b malignant lymphocytes , usually NK cells or, rarely, cytotoxic T cells. These cases, unlike other cases ENKTCL-NT that have more widespread disease, often show no or relatively little progression of their disease over long periods of time. From Wikipedia, the free encyclopedia. International Journal of Cancer.
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