Extranodal NK/T-cell lymphoma mimicking cellulitis.

NK/T-cell lymphoma is difficult to diagnose because there is no characteristic cytology to help the diagnosis in tissue sections, particularly when there is polymorphic cellular infiltration in the early stage of the disease. However, the nasal type of extranodal NK/T-cell lymphoma has a characteristic histologic pattern, which is angiocentric, angioinvasive and angiodestructive. Therefore, many cases of this tumor may show extensive necrosis that mimics infectious process. Furthermore, because the immunosuppressive status of these patients, they may, in fact, have superimposed infections. We are reporting a case that presented as cellulitis and only after careful examination with immunohistochemistry that a correct diagnosis of extranodal NK/T-cell lymphoma, nasal type, was established. Since this lymphoma is incurable and immunophenotyping is instrumental for the diagnosis and prediction of the prognosis, a high index of suspicion for this tumor is needed when an angiocentric lesion is found in the midline of the head and neck region, and a thorough immunohistological study should always be conducted in these cases.

Midline lethal granuloma--a clinical enigma.

Midline Lethal granuloma is characterized by progressive destruction of nose, paranasal sinuses and palate. Till date, the diagnosis of this mutilating process remains as enigma due to the non specific histological and systemic findings. However, over the years the clinicians have been able to divide the "Lethal midline granuoloma syndrome" into clinical entities: Idiopathic midline destructive disease, Wegener's granulomatosis, polymorphic retiaculosis and Non-Hodgkins lymphoma. This article attempts to distinguish between these disease entities in the light of 2 case reports of Idiopathic midline destructive disease.

Idiopathic midline destructive disease--does it exist?

There is controversy whether idiopathic mid-line destructive disease remains a valid entity, some authors considering that such cases are unrecognized malignant lymphoma or Wegener's disease. A case is presented in which numerous nasal biopsies over several years failed to show evidence of either lymphoma or Wegener's disease. Molecular genetic analysis of archival tissue did not demonstrate a monoclonal lymphoid population. It is proposed that idiopathic mid-line destructive disease remains a valid description in a small number of cases.

Angiocentric lymphomas of the palate: clinico-pathological considerations in 12 cases.

Angiocentric lymphomas (AL) involving the mid-face are infrequent lesions. Their incidence seems to be higher in certain Asian and Latin American countries, where most of the reported series reveal a predominance of T-cell lineage and a high frequency of EBV-positive cells. The clinical and pathological features of 12 AL that affected the palate are presented. Immunophenotypic studies were performed in nine of these cases. Seven (77%) demonstrated T-cell lineage, one was of B-cell origin and one could not be classified. The high incidence of T-cell lesions in this and other studies supports the existence of geographical and possibly of etiological differences in these neoplasms.

Lesión inmunoproliferativa angiocéntrica / Angiocentric immunoproliferative lesion

La lesión inmunoproliferativa angiocéntrica se propuso como un término alternativo para todas las lesiones linfoides que producen síndrome de granuloma letal de la línea media y se caracterizan histológicamente por infiltrado linfoie atípico, angiocéntrico y angiodestructivo, acompañado de necrosis y destrucción de las estructuras mediofaciales. La lesión inmunoproliferativa angiocéntrica incluye el granuloma de Stewart, la granulomatosis linfomatoide, la reticulosis polimorfa y el linfoma angiocéntrico. En este artículo, se presenta una revisión de la historia e histopatología de estas entidades, que han sido descritas bajo diferentes términos

Origin of malignant centrofacial granulomas: surface markers and gene rearrangement of malignant cells.

Malignant centrofacial granuloma (MCFG) is a clinical entity characterized by a relentless ulceration of the upper airway involving the nose, palate, and face, without any demonstrable etiology. The origin of 11 cases were analyzed with the help of cell-surface immunostaining in all and with T-cell receptor gene (TCR) rearrangement in 3. The results show that most of the cases of MCFG are in fact T-cell lymphomas with cell-surface antigens (CD2, CD7, CD3) consistent with either early or mature T lymphocytes. However, some cases exhibit B-lymphoid (CD19, CD20) or histiomonocytic (CD13, CD14) lineage-specific markers. In conclusion, despite its remarkable clinical unity, MCFG is a heterogeneous group of neoplastic diseases, most but not all of which may be classified as T-cell lymphoma.

[Nosologic criteria for the classification of midfacial granulomatosis]. / Critères nosologiques de classification des granulomatoses médio-faciales.

The authors report three cases of Stewart's disease and five cases of Wegener's disease seen over a period of 10 years, during which time they saw no cases of non-Hodgkins malignant lymphoma presenting as a mid-facial malignant granuloma. On the basis of these cases together with a review of the literature, they argue in favour of the dissociation of Stewart and Wegener as well as that of Stewart and monomorphous NHML, raising the problem of the existence of the latter as an entity.

Radiotherapy of polymorphic reticulosis.

The term "lethal mid-line granuloma" does not properly reflect current knowledge that several different diseases can produce non-infectious mid-facial destruction. The disorders producing erosion of the upper aerodigestive passages are more accurately classified by clinical and histological criteria as either Wegener's or non-Wegener's causes. The non-Wegener's causes of mid-facial erosion include extranodal lymphoma, idiopathic mid-line granuloma and polymorphic reticulosis. Polymorphic reticulosis (PR) is a rare disease which is histologically identical to lymphomatoid granulomatosis (LG). The term PR has been used to describe LG localized to the upper airways. PR-LG is characterized by a remarkably polymorphic angiocentric mixed cellular infiltrate. The disease is locally invasive, has the potential for distant spread, and can cause fever, sweats and weight loss. When it is localized to the upper airways, PR-LG is potentially controllable by radiation therapy. Local symptoms at other body sites can be successfully palliated by radiation. The clinical presentation of two recent patients with PR-LG is summarized, as well as the radiotherapy employed. The pertinent literature is reviewed.

Midline granuloma syndrome: a clinicopathologic study of 13 patients.

Thirteen patients with the clinical features of the midline granuloma syndrome are reported. Seven of the patients were determined to have Wegener's granulomatosis and had segmental necrotizing glomerulonephritis in their renal biopsies. Eighteen upper aerodigestive trace mucosal biopsies were available for review from the seven patients, and nine of these biopsies had a granulomatous angiodestructive inflammatory cell infiltrate considered "diagnostic" of Wegener's granulomatosis. The remaining nine biopsies lacked the specific histologic features of Wegener's granulomatosis but were considered consistent with mucosal involvement by the disease. Five of the remaining six patients had upper aerodigestive tract biopsies that were characterized by lymphocytic infiltrates. Three of the five patients had appreciable numbers of "atypical" cells in their biopsies and presented with radiologic evidence of lung involvement. It is our impression that patients with "significant cellular atypia" in their lymphocytic infiltrates have a disease indistinguishable from lymphomatoid granulomatosis, and these patients have a high propensity for either the presence or development of systematic disease that may require chemotherapy. Two patients had lymphocytic infiltrates with only minor degrees of cytologic atypia and no evidence of multisystem disease, and both of these patients responded to local radiation therapy. The remaining patient had a nonspecific histologic pattern in her numerous biopsies and was diagnosed as idiopathic midline destructive disease. She also had an adequate response to radiation therapy.

Wegener's granulomatosis and polymorphic reticulosis--two diseases or one? Experience with 90 patients.

Our experience with 50 patients with Wegener's granulomatosis and 40 patients with polymorphic reticulosis allowed us to compare these two disorders both clinically and histopathologically. Although these conditions are similar in some respects, they are separate and separable entities. The histologic features may be the most useful criteria for differentiation. Wegener's granulomatosis is characterized by the presence of vasculitis and necrotizing granulomas, whereas polymorphic reticulosis is typified by a mixed lymphoid infiltrate with an angiocentric growth pattern. The physician should be aware of the differences between these two disorders and should understand the implications of both conditions.

Non-healing granulomas of the nose.

Our knowledge and understanding of non-healing granulomas of the nose are from complete. The classification and etiology remain in on-going controversy. This article describes an uncommon case of Wegener's granulomatosis in childhood presenting with granulomatous involvement of the nose and orbit. A review of the literature is made with regard to non-healing granulomatous disease of the nose and some of the controversies in classification are pointed out.

Idiopathic pleomorphic midfacial granuloma (Stewart's type).

Idiopathic mid-facial granuloma is an unusual highly destructive disease which was virtually unknown prior to 1955. Great confusion exists, due in large part to the terminology applied to this entity as well as the clinical similarity of the process to other destructive mid-facial lesions. A classical case of idiopathic mid-facial granuloma of Stewart's type is presented, with characteristic lesions limited to the mid-face. Definitive histopathological features consist of pleomorphic inflammatory granulation tissue with patchy necrosis and no system lesions or neoplasia in the autopsy tissues.