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PubMedDate not supplied

[Lethal midline granuloma].

Lethal midline granuloma is a rare clinical syndrome. In clinical practice the destructive process of the facial midline may appear as a symptom of various infective, malignant or autoimmune diseases. A physician must have a good knowledge of the problem in order to make a rational approach to diagnosis. The present paper discusses the case of a 34-year old patient with destructive changes of midline, nose, perforating of palatal cleft and destruction of bone structure of nose, maxillary and ethmoid sinus. For histopathologic diagnosis of T-lymphoma it was necessary to make immunohistological study of the biopsy specimen. Irradiation therapy with total dose of 5600 cGy showed an extremely good therapeutic result. Three years after irradiation therapy the patient is still in a remission.

Adult↗
PubMed1997

Angiocentric T-cell lymphoma presenting as lethal midline granuloma.

BACKGROUND: Lethal midline granuloma (LMG) is a rare condition characterized by rapidly progressive midfacial destruction. Most LMG cases are angiocentric T-cell lymphomas and an association with Epstein-Barr virus (EBV) has been reported. Cutaneous involvement is poorly described and the prognosis not well documented. METHODS: We report a case of angiocentric T-cell lymphoma of the palate that presented as LMG with concurrent discrete skin lesions composed of two distinct morphologic appearances: indurated nodules and annular plaques. The English language literature for LMG-type angiocentric T-cell lymphoma is reviewed and a survival analysis of 58 cases with follow-up data (including our own case) is performed. RESULTS: The 1- and 5-year survival rates were 45% +/- 7% and 22% +/- 9%, respectively. Poor survival was associated with advanced age and stage. EBV DNA was detected in 16 out of 21 reported cases in which it was sought (including our case). CONCLUSIONS: We present photographic documentation of a broader spectrum of cutaneous lesions in the LMG-type angiocentric T-cell lymphoma than has previously been described, and have confirmed the association with EBV. The prognosis is poor. Aggressive therapy such as bone marrow transplantation should be considered early in the course.

Age Factors↗
PubMed1989

Current concepts of the lethal midline granuloma syndrome.

"Lethal midline granuloma" is an unusual but devastating spectrum of lymphoproliferative disorders that is now thought to include four entities: idiopathic midline destructive disease, Wegener's granulomatosis, polymorphic reticulosis, and nasal lymphoma. These disorders must be considered in the differential diagnosis of any patient with chronic, nonspecific complaints localized to the upper airway. An aggressive diagnostic and therapeutic approach may significantly decrease morbidity and death resulting from these lesions.

Aged↗
PubMed1994

[Differential diagnostic aspects of lethal midline granulomas (granuloma gangraenescens].

The lethal midline granuloma and limited Wegener's granulomatosis show clinical similarity, although they are of different etiology. The following case of a 53-year-old woman shows how difficult it is to establish the precise diagnosis of lethal midline granuloma. The diagnosis depends on the pathological finding of a lymphoma. The lymphoma can be differentiated in a T- or a B-cell lymphoma by immunostaining. However, the diagnostic yield of biopsies from the nose is not perfect. It would be, therefore, important to find other diagnostic criteria. The presence of the c-ANCA is a helpful tool, but in the case of limited Wegener's granulomatosis, it has a sensitivity of 50%. The prognosis of the lethal midline granuloma is poor even if an adequate radiation therapy is instituted.

Diagnosis, Differential↗
PubMed2006

Prosthetic rehabilitation of a midfacial defect resulting from lethal midline granuloma--a clinical report.

Prosthetic rehabilitation of the midfacial defects has always perplexed maxillofacial prosthodontists. These midfacial defects lead to functional and cosmetic deficiencies. One of the causes of such defects is midline lethal granuloma, which is a destructive granulomatous lesion of uncertain aetiology, involving the nose, paranasal sinuses and the palate. Presented here is a clinical report of a 22-year-old male patient diagnosed to have lethal midline granuloma, who was referred to the Department of Prosthodontics, SDM College of Dental Sciences Dharwad. The patient had a severe midfacial defect involving the nose, the paranasal sinuses, the palate and the soft tissues of the face. Although the lesion was not of the rapidly progressive type at the time of rehabilitation, the non-specific inflammatory process was persisting. The patient was treated with chemotherapy and palliative therapy. The primary objective of the prosthetic rehabilitation was to provide closure of the severe defect to protect the soft tissues from environmental exposure. Secondarily the prosthesis also provided acceptable aesthetics and psychological benefit to the patient.

Adult↗
PubMed1984

[The role of radiotherapy in the treatment of granuloma gangraenescens (lethal midline granuloma].

The granuloma gangraenescens in the oral, maxillary and facial region is a rare disease. It is a destroying process in the region of palate, nose, paranasal sinuses, cheeks and orbit with characteristic signs of granulomatosis, infection and malignancy. The disease shows often a lethal development with cachexia or sepsis. Besides local inflammations and tumors, the granulomatosis of Wegener, and the so-called necrotizing sialometaplasia are above all to be excluded by differential diagnosis. Five cases are presented in order to describe the diagnostic and therapeutic problems. In literature, the greatest efficacy is attributed to radiotherapy, however, a detailed definition of the most efficient irradiation conditions cannot be given yet because of the small number of cases. Good long-term results or recoveries can be achieved in 75 to 80% of cases by a relatively high radiation dose of 40 to 50 Gy administered within four to five weeks. During the observation time of two to six years, no one of the five patients treated here only by megavoltage therapy showed a recurrence.

Adult↗
PubMed1992

[Genotypic analysis of lethal midline granuloma].

So-called lethal midline granuloma is of great clinical and theoretical interest. The etiology of lethal midline granuloma is unknown and the pathogenesis is variable, with debate as to precise classification and natural history. In this study, we reported genotypic and immunopathological features in 3 cases of lethal midline granuloma. The histopathological diagnosis of their biopsy specimens was initially polymorphic reticulosis/midline malignant reticulosis. Immunohistologic study of the specimens revealed that immature or atypical cells had phenotypes of T-cells, CD2, CD3, CD4 (Case 1), CD4 (Case 2), and CD2, CD3 (Case 3). Those cells were also found to be positive for HLA-DR, which indicated that they were activated T-cells. Immunohistology in T-cells, however, was not able to give a similar clue to clonarity as it was possible within B-cell neoplasms by immunophenotyping the light chains. With the establishment of cDNA probes for the T-cell receptor genes it was possible to analyze neoplasms of lymphocyte origin for lineage and clonality. The Southern blot analysis of 3 cases showed rearrangement of TCR gene, TCR beta and TCR gamma chain (Cases 1 and 2) and TCR beta and TCR delta chain (Case 3), whereas none of them showed rearrangement of immunoglobulin heavy chain. These findings represented conclusive evidence for a monoclonal T-cell proliferation within lethal midline granuloma. On the ground of immunohistological and genotypic studies, lethal midline granuloma histologically diagnosed as polymorphic reticulosis/midline malignant reticulosis are proven to be a T-cell lymphoproliferative disorder.

Adult↗
PubMed1988

Lymphomatoid granulomatosis--evidence of a clonal T-cell origin and an association with lethal midline granuloma.

Lymphomatoid granulomatosis and lethal midline granuloma are both characterized histologically by atypical pleomorphic angiocentric infiltrates. Whether these conditions are malignant lymphoproliferative disorders remains controversial. Here we report the results of studies carried out in a patient with coeliac disease, who developed recurrent self-healing subcutaneous nodules with the histological changes of lymphomatoid granulomatosis and an invasive nasal tumour with the histological features of lethal midline granuloma. The patient subsequently also developed an erythrophagocytic syndrome. Immunocytochemical labelling of both cutaneous and nasal lesions demonstrated a predominant population of T cells with a CD4-negative CD8-positive phenotype. Analysis of DNA from cutaneous tissue revealed a discrete rearrangement of the beta and gamma T-cell receptor genes. These findings suggest that lymphomatoid granulomatosis is a clonal T-cell lymphoproliferative disorder and its association with lethal midline granuloma indicates that both conditions may have a common histogenesis.

Celiac Disease↗
PubMedDate not supplied

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.

Adult↗
PubMed1990

Epstein-Barr virus in nasal T-cell lymphomas in patients with lethal midline granuloma.

Five cases of lethal midline granuloma were identified histologically and phenotypically as peripheral T-cell lymphomas. Epstein-Barr virus (EBV) DNA was detected in the nasal tumour biopsy specimens by Southern blotting and in-vitro hybridisation with simultaneous detection of EBV-determined nuclear antigen (EBNA) and T-cell surface markers by two-colour immunofluorescence. Further immunofluorescence and northern blotting revealed that EBNA2 gene and also latent membrane protein gene were expressed in the nasal tumour cells. The patients had high titres of antibodies to EBV. These findings suggest that lethal midline granuloma is causally associated with EBV.

Adult↗
PubMed2003

Midline lethal granuloma complicating pregnancy: case report.

A case of midline lethal granuloma in a 28-year- old female Nigerian patient is reported. Oral, ocular and nasal lesions were present and these preceded a spontaneous abortion of a three month old pregnancy. The clinical course of the disease and its similarity to other granulomatous diseases, which are generally classified as midline granuloma syndrome, are highlighted. The prognosis is poor but early diagnosis and treatment appears to improve a patient's condition

Abortion, Spontaneous↗
PubMed1976

Lethal midline granuloma: a pathological spectrum.

Lethal midline granuloma is a non-specific clinical term used for different pathological entities often exhibiting some semblance to each other as they lie along a histological spectrum. Nevertheless, each entity has a specific diagnostic pattern. The histological picture with distinguishing features of each specific type is pointed out. Stress is made on the use of repeated biopsy until the specific histological diagnosis can be made so that specific treatment as breifly outlined, can be carried out to insure the best prognosis.

Biopsy↗
PubMed1990

[Contribution of immunologic technics to the characterization of lethal midline granuloma of unknown origin].

The lethal midline granuloma is a clinical entity characterized by a relentless ulceration of the upper airway involving the nose, the palate and the face, without any demonstrable etiology. We have applied the cell membrane immunostaining techniques to twelve cases. According to the results, it seems that most of the cases are in fact T-cell lymphomas with membrane staining consistent with either precursor or mature lymphoid T-cells. Some cases, however, exhibit an immunostaining pattern compatible with other origins, the proliferating cells belonging either to the B lymphoid or to the histio-monocytic lineages. We conclude that the lethal midline granuloma is an heterogeneous group of neoplastic diseases, in the most part close to a T cell lymphoma, but with a remarkable clinical unity.

Granuloma, Lethal Midline↗
PubMed2000

Lethal midline granuloma: report of three cases.

Lethal midline granuloma (LMG) is a rare clinical entity characterized by progressive relentless ulcerations and necrosis of midfacial structures. It occurs more frequently in Oriental than in Western populations with no demonstrable etiology. Treatment and outcome for cases differ, but their pathological distinction may not always be possible from routine biopsy specimens. The histological features often seen include widespread coagulative necrosis, heavy inflammatory infiltrates, and atypical pleomorphic cells. However, the paucity of these atypical cells in biopsy specimens and the degree of necrosis can make the diagnosis of a neoplastic lesion very difficult. Because of the progress in pathology methodology including immunohistochemistry, most cases have been proven to be malignant lymphomas of T-cell lineage. We present 3 patients for whom an initial clinical diagnosis of LMG was made. From their several oral biopsies and nasal specimens, difficulties were encountered in differentiating "midline granuloma" from other possible diseases using histomorphological criteria alone. After extensive evaluations, malignant T-cell lymphoma was the specific disease entity identified in only one case by cell membrane immunostaining technique. A literature review was carried out, and recent concepts of the etiology and pathogenesis of this disease are presented.

Adult↗
PubMed1996

[Two cases of lethal midline granuloma thought to be of natural killer cell origin].

So called lethal midline granuloma is of great clinical and theoretical interest. Recent evidence has shown that most lethal midline granulomas are associated with a T-cell phenotype and they are therefore referred to as nasal T-cell lymphomas (NTCL). Immunohistochemical studies, however, have shown peculiar phenotypic features such as expression of natural killer (NK)-cell-related markers and extensive T-cell antigen loss including absence of expression of alpha beta T-cell receptor (TCR). In this study, we reported genotypic and immunohistochemical features in two cases of lethal midline granuloma. The histopathological diagnosis of the biopsy specimens was polymorphic reticulosis/midline malignant reticulosis. Both cases displayed a CD2+, CD3-, CD3 epsilon+, CD4-, CD8-, CD16-, CD56+ phenotype, suggesting that these tumors may be peripheral T-cell lymphomas with extensive loss of T-cell antigens and expression of NK cell antigen (CD56), or, alternatively, NK cell neoplasias. No TCR beta gene rearrangement was detected in these cases. Monoclonal Epstein-Barr virus (EBV) genome was detected in each specimen by Southern blot hybridization. The tumor cells in one of the two cases expressed latent membrane protein (LMP). These findings support the concept that lethal midline granuloma constitutes a distinct group of lymphomas that, in addition to their peculiar clinical features, exhibits the phenotype of extensive loss of T-cell antigens and expression of the NK cell antigen, as well as harbors the EBV. In view of the LMP-transforming potential, these data suggest that EBV may play a role in the pathogenesis of lethal midline granuloma.

Aged↗
PubMed1975

[Midline malignant reticulosis (so-called lethal midline granuloma) (author's transl)].

Case report about a 30-year old man with midline malignant reticulosis. Diagnosis was not successful by means of biopsy but given by microscopic examination of autopsy material afterwards. So-called lethal midline granuloma is a clinical term. After excluding specific infections, carcinomas or other rare diseases, it is caused by midline malignant reticulosis or by Wegener's granulomatosis. Frequently diagnosis is made by the pathologist at autopsy.

Adult↗