Adenocarcinoma developing in a patient with midline granuloma.

A 51-year-old man with midline granuloma was treated successfully with a combination of local radiation and oral cyclophosphamide. Six years after therapy, adenocarcinoma of the lung developed. Possible relationships between these two diseases are discussed.

Experience of radiotherapy in lethal midline granuloma with special emphasis on centrofacial T-cell lymphoma: a retrospective analysis covering a 34-year period.

Lethal midline granuloma (LMG) is characterized by progressive ulceration and destruction of the midfacial tissue. It occurs more frequently in Oriental than in Western populations. Because of the progress in clinical pathology and immunohistochemistry, most cases have been proven to be malignant lymphomas, especially of T-cell lineage. We describe 92 cases of lethal midline granuloma or centrofacial malignant lymphoma in the period 1959-1993. All received complete courses of radiotherapy. Twenty of them also received combination chemotherapy. Thirty-six cases had specimens available for immunohistochemical study; 25 (69%) of these had a T-cell phenotype, and 6 (17%) were of B-cell lineage. The dose to the nasal region was in the range of 3000-7500 cGy in 11-58 days, and to the neck 3000-6400 cGy in 11-48 days. The overall survival rate for the LMGs was 59.5% at 5 years and 56.2% at 10 years (Kaplan-Meier). Combined chemotherapy seemed not to improve the overall survival in this study (p = 0.63), but the patient number was too small to make a firm conclusion. Based on the results of this study, we recommend a dose of 4500-5000 cGy to the midfacial region, since a higher dosage did not improve the treatment results (p = 0.88). Irradiation has a definite role in good locoregional control of this disease. The recent clarification of the disease nature and the recognition of the background clinicopathological features should provide valuable information for future patient management and prospective studies.

Idiopathic midline destructive disease (IMDD): a subgroup of patients with the "midline granuloma" syndrome.

"Midline granuloma syndrome" encompasses a number of specific clinicopathologic entities, such as "Wegener's granulomatosis, midline malignant reticulosis, lymphoma, nasal carcinoma and a broad spectrum of infectious diseases. In the course of studying a number of patients presenting with destructive lesions of the upper respiratory tract, 11 cases emerged as having unique clinicopathologic features. These are: 1) presence of locally destructive lesions which are always restricted to the upper respiratory tract. 2) Absence of systemic disease during a follow-up period of six months to 18 years (mean 7.3 years). On examination of repeated biopsy specimens the histopathologic picture consisted of acute and chronic inflammation with variable amounts of necrosis. Granulomata were seen in one case. Malignant or atypical cells were invariably absent. Frank vasculitis was not seen, although in five of the patients inflammatory cells in the wall of small vessels were conspicuous 4) Inability to demonstrate an infectious origin by culture or special stains. Radiation therapy has proven effective in patients with this entity, for which the term "Idiopathic Midline Destructive Disease" (IMDD) is proposed.

Midline granuloma.

A 34-year-old woman had a granulomatous ulceration of the nose that responded to radiation therapy. The limitation of the disease to the nose and the histologic findings were consistent with a diagnosis of midline granuloma, Stewart type. The relationship of midline granuloma to Wegener's granulomatosis is reviewed. In view of the uniform mortality that accompanies untreated midline granuloma, a correct diagnosis must be made so that radiation therapy can be promptly instituted.

The protean clinical features of polymorphic reticulosis (lethal midline granuloma).

Confusion surrounds the entity known as "lethal midline granuloma". Partly responsible is the lack of specificity in this term. "Polymorphic reticulosis" has been used as a term to describe the morphology of the disease. Thirty-two cases illustrate the protean features of this disease. Although it commonly presents in the head and neck, other sites such as the lungs, kidneys, skin, and gastrointestinal tract may be involved, either alone or in conjunction with lesions of the head and neck. Clinically, it is easily confused with Wegener's granulomatosis. Histopathologic differentiation, however, is both feasible and important. Wegener's granulomatosis is treatable with steroids with or without cyclophosphamide; polymorphic reticulosis confined to one site responds to irradiation. In polymorphic reticulosis, thhe best results of treatment are obtained in localized lesions of the upper airway treated early with irradiation; a poorer outcome is associated with multifocal involvement, which necessitates systemic therapy.

Nasal peripheral T-cell lymphoma: a 20-year review of cases treated in Scotland.

The nasal peripheral (post-thymic) T-cell lymphoma is an important cause of the midline granuloma syndrome (MGS), in which ulceration and destruction of the tissues of the nose and paranasal sinuses occurs. We reviewed the histology of 9 cases of the MGS treated with radiotherapy, and, using immunocytochemistry, showed 8 cases to be peripheral T-cell lymphomas (PTCL) and 1 a B-cell lymphoma. All patients received radiotherapy and 2 died shortly after treatment from unrelated causes. Two patients with T-cell lymphoma and the solitary case of B-cell lymphoma achieved long-term disease-free survival. The 4 remaining cases of T-cell lymphoma relapsed locally at a median interval of 3.5 months despite megavoltage irradiation of 45-50 Gy (in 3 cases) and inclusion of uninvolved paranasal sinuses and the nasopharynx in the field (in 2 cases). All patients with local relapse achieved, and remain in, remission after treatment with alkylating agents and prednisolone. The disappointing response of some cases of nasal T-cell lymphoma to radiotherapy has been reported by others, and this may be due partly to the heterogeneity of nasal lymphomas. We are unable to provide clear guidelines for treatment but suggest that a role exists for initial treatment with oral alkylating agents and steroids in newly diagnosed cases.

Treatment of "idiopathic midline destructive disease" by irradiation. A case report.

An unusual case with an aggressive destructive granulomatous lesion of the maxilla is reported. Although the possibility of infection and neoplasm could be ruled out, a definite diagnosis could not be established even by repeated biopsies which showed the lesion to be a non-specific inflammatory process. Since Wegener's granulomatosis was most unlikely, the patient was treated by radiotherapy which caused rapid remission of the lesion with no sign of recurrence after 4 years. The clinical and histological findings as well as the responsiveness to the treatment were most indicative of idiopathic midline destructive disease. Effective management of lethal midline granuloma of unknown aetiology is discussed.

Steward's midline granuloma. A case report.

Steward's type of neoplastic disease is rare. Much has been discussed on the histopathology of this granuloma. Most authors define it as a neoplasm of the lymphoid system, and it is a subgroup of the "midline granuloma syndrome". We report a case of a 19-year-old first treated with chemotherapy (cyclophosphamide and prednisone) and then with radiotherapy, with good results.

Lethal midline granuloma starting as granuloma laryngis.

We report a case of lethal midline granuloma in a 34-year-old male patient. The patient was referred to our hospital because of long-lasting hoarseness. He was treated for granuloma laryngis. After two years nasal obstruction developed followed by ulceration of the hard palate and destruction of part of the nose and the upper lip. A biopsy demonstrated polymorphic infiltrate consisting of small lymphocytes, plasma cells, macrophages, atypical lymphoid cells and eosinophils. Radiotherapy was very effective and led to long-term remission. The different etiological aspects of this syndrome are discussed.

Midline malignant reticulosis.

A combined histological, ultrastructural and cytological study was performed on biopsy materials from nasal lesions of 2 patients with midline granuloma. The histological and ultrastructural features of the tissues were necrosis, inflammation and infiltration of atypical histiocytic cells. The DNA-content of these histiocytes exceeded the limits of the 5c Exceeding-Rate and the 2c Deviation Index. The morphological characteristics and the results of the DNA-cytophotometry classify the midline granuloma as a local malignant histiocytosis. Clinical follow-up of the cases suggests that radiation therapy offers a good prospect of treatment success.

Malignant histiocytosis (histiocytic sarcoma). A (the?) major cause of the 'midline granuloma syndrome'.

Five out of eight consecutive cases with initial symptoms of a 'midline granuloma' were identified as malignant histiocytosis (histiocytic sarcoma) which within 5 months to 4 years led to generalization and death. The three remaining cases also fulfilled the morphological criteria of this type of neoplasia, though these patients are still alive 1/2 to 8 years after diagnosis, possibly as a result of local radiotherapy. The age of the individuals ranged from 18 to 71 years and there was a male preponderance of 7:1. The histiocytic nature of the atypical cells was primarily documented by intense activity of NaF-inhibitable non-specific esterase, of acid phosphatase and of beta-glucuronidase as demonstrated in cryostat sections of formaldehyde-saccharose-fixed fresh biopsy specimens and by the detection of alpha-1-antichymotrypsin, alpha-1-antitrypsin, and lysozyme antigens, in that order of constancy (immunohistochemical examination of formaldehyde-fixed paraffin sections, using the avidin-biotin-peroxidase complex method). There was among the reported cases a considerable heterogeneity with regard to these 'markers'. We conclude that malignant histiocytosis is a (the?) major cause of the 'midline granuloma syndrome'.

[Is Stewart's malignant midline granuloma a peripheral T-cell lymphoma? Apropos of 5 cases]. / Le granulome malin centro-facial de Stewart est-il un lymphome T périphérique? A propos de 5 observations.

Midline granuloma includes diverse clinicopathological entities, such as Wegener granulomatosis, polymorphic reticulosis, lethal midline granuloma and conventional malignant lymphoma of the nose usually of B-cell origin. The authors describe five patients with LMG clinically and pathologically typical. Using an extensive panel of monoclonal antibodies, they demonstrate an "activate" T-cell phenotype observed on the initial lesion of the face in one patient, similar to that found in two patients with LMG but studied after dissemination in peripheral T-cell lymphoma. Furthermore, many atypical cells were found in LMG, and stained with the Ki-67 monoclonal antibody, a marker of proliferating cells. These findings support the view that LMG is closely related to T-cell malignancies. Two of them were treated with recombinant Interferon alpha 2a followed by a response rapidly objective. Immunohistologic studies are very important for confirming the T-cell origin of such a disease and for selecting patients to be treated with Interferon alpha.

[Weiss' nasal lymphoma (histiocytic, malignant)]. / Linfoma nasal de Weiss (histiocítico-maligno).

We presented a patient with a deforming and necrotizing syndrome of the nasal pyramid. The histopathology demonstrated a lymphoma. The original works of Weiss are related. The diseases is characterized by: ecotaxis of the nasal pyramid, localized persistency during a long time, malaise in advanced studies and sensibility to radiations. We proposed the name linfoma histiocitico maligno nasal de Weiss and consider it as a autonomous entity.