phenylephrine-hydrochloride and Granuloma--Lethal-Midline

We describe a 36-year-old patient with an aggressive, midline intranasal and naso- and oropharyngeal destructive process. For months the patient denied heavy abuse of nasal cocaine, but finally admitted it. Necrosis and atrophy of the inferior and middle nasal turbinates bilaterally, prominent naso and oropharyngeal ulcers, nasal septal as well as hard palate perforation were observed clinically. Repeated biopsies revealed focal areas of chronic inflammation and necrosis, but there was no evidence of vasculitis or granuloma formation. Since serum was slightly positive for antineutrophil cytoplasmic antibody, the initial diagnosis was Wegener's granulomatosis. In the United States there have been a few reports on a new cocaine-associated syndrome presenting as an aggressive, midline, intranasal and intrapharyngeal destructive process mimicking limited Wegener's granulomatosis and midline reticulosis. We report the first such case in Europe and offer guidelines for the diagnostic work-up of such cases.

Topics: Adult; Biopsy; Cocaine-Related Disorders; Diagnosis, Differential; Disease Progression; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Male; Necrosis; Nose; Palate

Topics: Blood Protein Electrophoresis; Cilia; Diagnosis, Differential; Granuloma; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Herpes Simplex; Humans; Kidney Transplantation; Leishmaniasis, Mucocutaneous; Leprosy; Lysosomes; Microscopy, Electron; Mycoses; Nasal Polyps; Nose; Postoperative Complications; Prognosis; Respiratory Tract Diseases; Rhinitis; Rhinoscleroma; Sarcoidosis; Syphilis; Transplantation, Homologous; Tuberculosis; Tuberculosis, Laryngeal

We present history, clinical presentation and anatomo-pathologic findings of a 24-year-old female patient with a nasal NK/T-cell lymphoma. This rare tumor is characterized by its angiocentric and angiodestructive growth, which results in extensive tumor necrosis. At the first encounter this tumor necrosis made it difficult to identify the nature of the tumor cells. However, this necrosis is a key feature: it is the result of the capacity of neoplastic NK/T-cells to invade vessels. The T-cell character of the neoplastic lymphoid has been shown by immunohistochemitry.

Topics: Adult; Diagnosis, Differential; Fatal Outcome; Female; Granuloma, Lethal Midline; Humans; Killer Cells, Natural; Lymphoma, T-Cell; Nose; Nose Neoplasms

Topics: Adult; Combined Modality Therapy; Fatal Outcome; Granuloma, Lethal Midline; Humans; Immunophenotyping; Karyotyping; Killer Cells, Natural; Lymphoma, T-Cell; Male; Neoplasm Recurrence, Local; Nose; Nose Neoplasms

Topics: Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; History, 20th Century; Humans; Kidney; Lymphomatoid Granulomatosis; Nervous System; Nose; Scotland; Urethra

Destructive lesions of the sinonasal tract, lacking a discernible etiology and referred to as midline destructive disease, have been pathologically classified in accordance with a variety of confusing terms. Development of new pathologic concepts and immunohistochemical techniques has provided a fresh understanding of these lesions, and, as a result, they can be unified into two distinct pathologic groups: Wegener's granulomatosis and non-Hodgkin's T-cell lymphoma.. We retrospectively reviewed the imaging studies and pathologic specimens of seven patients with prior diagnoses included in the midline destructive disease group. The specimens were reviewed by an oral pathologist using currently accepted pathologic criteria and the newly available immunohistochemical markers CD20, CD45, and CD45RO. Lesions were classified as non-Hodgkin's T-cell lymphomas when positive for CD45 and CD45RO and negative for CD20, and as Wegener's granulomatosis in the presence of noncaseating multinucleated giant cell granulomas and necrotizing vasculitis.. Three of the lesions were reclassified as Wegener's granulomatosis and four as T-cell lymphomas after applying these pathologic criteria. There were no distinguishing imaging findings between Wegener's granulomatosis and non-Hodgkin's T-cell lymphoma.. The current pathologic classification for midline destructive disease should be incorporated into the radiologic lexicon and the use of terms from the old classification system, such as idiopathic midline granuloma and lethal midline granuloma, should be abandoned and no longer be used in radiologic reports.

Topics: Antigens, CD20; Diagnosis, Differential; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Leukocyte Common Antigens; Lymphoma, T-Cell; Magnetic Resonance Imaging; Nose; Nose Diseases; Nose Neoplasms; Retrospective Studies; Sensitivity and Specificity; Terminology as Topic; Tomography, X-Ray Computed

Extranodal non-Hodgkin's lymphoma in the head and neck, especially T-cell lymphoma of the lethal midline granuloma (LMG) type, has unique clinical and histologic features differentiating it from other lymphomas. The authors measured soluble intercellular adhesion molecule-1 (ICAM-1) in sera from 12 patients with T-cell lymphoma of the LMG type and from 52 patients with other head and neck non-Hodgkin's lymphomas, by double-determinant immunoassay. The expression of ICAM-1 in lymphoma tissue was examined in 26 patients by the avidin-biotin immunoperoxidase method. The serum ICAM-1 levels were significantly higher in T-cell lymphoma of the LMG type than in other head and neck lymphomas or in healthy adult controls. Elevated levels of serum ICAM-1 were associated with increased levels of serum lactate dehydrogenase, poor prognosis, and impaired T-cell-dependent immune functions, both in T-cell lymphoma of the LMG type and in other head and neck lymphomas. When we monitored serum ICAM-1 levels in individual patients, the level decreased in the complete remission interval compared to before treatment and went up again when the lymphoma relapsed. Although the staining intensities of ICAM-1 in lymphoma cells were not related to serum ICAM-1 levels, a markedly intense expression of ICAM-1 was found on the angiocentric or angiodestructive lymphoreticular infiltrate region in the tissues from T-cell lymphoma of the LMG type. A higher serum ICAM-1 level and its tissue expression in T-cell lymphoma of the LMG type may be one of the clues to understanding this particular lymphoma. The serum ICAM-1 level could be an efficient parameter for monitoring the clinical course of head and neck non-Hodgkin's lymphomas.

Topics: Adult; Aged; CD4 Antigens; CD8 Antigens; Female; Granuloma, Lethal Midline; Humans; Intercellular Adhesion Molecule-1; L-Lactate Dehydrogenase; Lymphoma, Non-Hodgkin; Male; Middle Aged; Nose; Nose Neoplasms; Paranasal Sinus Neoplasms; Paranasal Sinuses

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.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; B-Lymphocytes; Cell Lineage; Child; Combined Modality Therapy; Disease-Free Survival; Facial Neoplasms; Female; Follow-Up Studies; Granuloma, Lethal Midline; Humans; Immunohistochemistry; Immunophenotyping; Lymphoma, T-Cell; Male; Middle Aged; Neck; Nose; Radiotherapy Dosage; Radiotherapy, High-Energy; Retrospective Studies; Survival Rate; T-Lymphocytes; Treatment Outcome

For the understanding of the CT findings of polymorphic reticulosis involving the upper respiratory tract, we reviewed eleven cases of this disease with initial CT study before treatment. CT revealed masses or swelling in the nose or nasal chamber, often with involvement of paranasal sinuses and medial parts of the orbit. In some, nonspecific minimal bony change (either bone destruction or bone sclerosis) was found. These CT findings are not specific. Any large mass in the nose and nasal fossa with little associated bone destruction should, however, raise the differential diagnosis of polymorphic reticulosis.

Topics: Adolescent; Adult; Aged; Carcinoma, Squamous Cell; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Male; Middle Aged; Nasal Cavity; Nose; Nose Neoplasms; Tomography, X-Ray Computed

Topics: Aged; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Nose; Skin

Destructive processes of the midface can occur in a wide variety of diseases. Intrinsic in consideration of these is a cluster of lesions, including Wegener's granulomatosis (WG), idiopathic midline granuloma (IMG), polymorphic reticulosis (PR), and lymphoma. Although there is still confusion as to whether the latter three represent a spectrum of the same malignant process, there is general agreement that WG is a separate entity on the basis of clinical presentation and therapeutic response. It is probable that PR is an emergent lymphoma, with the same prognostic and therapeutic features. Idiopathic midline granuloma is clinically similar to PR and lymphoma, but histologically it appears to be inflammatory in nature with no clearly definable malignant cell type present. At this point in time three diseases are best collectively referred to as midline "nonhealing" granuloma. The cases presented represent the spectrum of this enigmatic process.

Topics: Adult; Connective Tissue; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Histiocytes; Humans; Leukocytes; Male; Maxillary Diseases; Nose

Biopsies of two patients with a clinical diagnosis of lethal midline granuloma were examined by electron microscopy, histology and cytology. Morphological changes included necrosis, inflammation, and infiltration of atypical histiocytic cells. Judging by the cellular characteristics, the DNA-content and -distribution pattern, this localised, invasive and destructive lesion is a low-grade malignant histiocytic tumour. Our results show that final tumour diagnosis can only be established by special cytological methods.

Topics: Connective Tissue; DNA; Female; Granuloma, Lethal Midline; Histiocytes; Humans; Microscopy, Electron; Middle Aged; Necrosis; Nose; Paranasal Sinuses

Topics: Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Nose; Terminology as Topic

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.

Topics: Adult; Aged; Biopsy; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Lymphomatoid Granulomatosis; Male; Middle Aged; Nose

The clinical and histologic materials, including autopsy findings, from 6 cases with clinical features of so-called lethal midline granuloma were examined. These cases were selected by characteristic biopsy findings observed in the nasal lesions. The histologic features of the nasal lesions were necrosis and infiltration of atypical histiocytic cells. The mean age was 37 years with a 2:1 male to female preponderance. Nasal complaints and fever were frequent manifestation, and physical findings showed necrotic, granulomatous lesions in the nasal cavity in all cases. The survival ranged from 4 to 24 months, giving a mean value of 13 months. Autopsy findings revealed systemic, neoplastic proliferation of atypical histiocytes, from these observation, these 6 cases should be regarded as malignant histiocytosis (MH) with usual presentation.

Topics: Adult; Biopsy; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Humans; Lymph Nodes; Lymphatic Diseases; Male; Middle Aged; Nose; Skin

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.

Topics: Face; Female; Granuloma, Lethal Midline; Humans; Middle Aged; Necrosis; Nose; Temporal Bone

Topics: Adult; Autopsy; Brain; Diagnosis, Differential; Facial Neoplasms; Female; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Kidney; Male; Nose; Staining and Labeling

Topics: Diagnosis, Differential; Eosinophilic Granuloma; Female; gamma-Globulins; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Middle Aged; Nose; Nose Diseases; Prednisolone

Topics: Adult; Autopsy; Cortisone; Granuloma, Lethal Midline; Humans; Male; Nose; Skin Transplantation; Time Factors; Transplantation Immunology; Transplantation, Autologous

Topics: Collagen; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Humans; Methylprednisolone; Microscopy, Electron; Middle Aged; Nose

Topics: Adult; Cortisone; Diagnosis, Differential; Face; Facial Neoplasms; Female; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Immunosuppressive Agents; Lymphoma, Non-Hodgkin; Male; Middle Aged; Nasal Mucosa; Nose; Nose Neoplasms

Topics: Adult; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Humans; Lymphatic Diseases; Male; Middle Aged; Nose

Topics: Cervix Uteri; Diagnosis; Female; Granuloma; Granuloma, Lethal Midline; Granulomatosis with Polyangiitis; Humans; Nose; Pathology; Vagina

Topics: Granuloma; Granuloma, Lethal Midline; Humans; Nose; Nose Diseases

Topics: Granuloma; Granuloma, Lethal Midline; Humans; Liver Neoplasms; Mesenchymoma; Nose

Topics: Granuloma; Granuloma, Lethal Midline; Humans; Nasopharynx; Nose