Extranodal natural killer/T-cell lymphoma, nasal type: 'midline lethal granuloma.' A case report.

Extranodal natural killer/T cell lymphoma, nasal type, is a non-Hodgkin lymphoma, most commonly affecting the nasal cavity, paranasal sinuses and nasopharynx. Clinically it is characterised by destruction of facial tissues, commencing in the midline. In most cases it arises from malignant transformation of natural killer cells (NK); sometimes from malignant transformation of cytotoxic T cells.Extranodal NK/T cell lymphoma, nasal type, is rare, but even more rare in black persons. The purpose of this article is to report a severe case of extranodal NK/T cell lymphoma, nasal type, in an elderly black male.

[Angiocentric T/NK cell lymphoma: a special clinical-pathological entity of lethal midline granuloma. A case report]. / Das angiozentrische T/NK-Zell-Lymphom--eine spezielle klinisch-pathologische Entität des letalen Mittelliniengranulomes: eine Fallbeschreibung.

BACKGROUND: The term "midline granuloma syndrome" (MGS) is a clinical description of a broad spectrum of diseases, which are characterised by aggressive and progressive destruction of mucosa and adjacent structures of the midface and upper aerodigestive tract. After exclusion of granulomatous infections, rare granulomatous diseases and epithelial neoplasias, the differential diagnosis includes the following entities: Wegener's granulomatosis (WG), malignant lymphoma and idiopathic midline destructive disease (IMDD). Today there are doubts about the existence of IMDD. After exclusion of WG nearly all remaining cases presenting as MGS are peripheral sinonasal angiocentric T- and/or NK-cell lymphomas, which show a close association to Epstein-Barr virus infection and now are recognised as a special clinicopathological entity. The natural history of these lymphomas is characterised through a rapidly progressive course with a poor prognosis. PATIENT: A case of a 35-year-old male patient with an angiocentric nasal T/NK-cell lymphoma, which involved the left lacrimal cyst, the left maxillar and ethmoid sinus as well as the soft and hard palates, is presented. First clinical signs and symptoms were similar to chronic-recurrent sinusitis. For almost two years the patient was treated with systemic corticoids for suspected limited Wegener's granulomatosis. The patient underwent sinus surgery for pansinusitis three times. After development of midline destructive disease the diagnosis of angiocentric lymphoma was established. RESULTS: Soon after the diagnosis a combination high-dose radiochemotherapy was performed. The patient died only 3 months later because of multiorgan failure. CONCLUSIONS: Because of its poor prognosis the angiocentric nasal NK/T-cell lymphoma should included early into the differential diagnosis of the midline granuloma syndrome. Correct biopsy technic and in situ hybridization of EBV can be important for an early diagnosis. Therapy should be aggressive and consists of high-dose radiotherapy, which is most important to reach local tumor control, and combination chemotherapy, the use of which is presently in discussion.

[Diagnostic efficacy of 18F-fluorodeoxy glucose-positron emission tomography in multiple solitary pulmonary nodules].

A 73-year-old man was admitted to our hospital for evaluation of abnormal lung shadows in the left lung. Chest computed tomography revealed a cavitary lesion with irregular edges in the right S10 and a nodular lesion with well-defined margins in the left S6. Histological examination of a specimen obtained by transbronchial lung biopsy revealed squamous cell carcinoma in the right S10 but no significant findings in the left S6. Thirdly, 18F-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) demonstrated that the nodular shadow in the left S6 was a low-uptake structure and that the cavitary lesion in the right S10 was a high-uptake lesion. We therefore considered that the nodular shadow in the left S6 was not one of neoplastic disease. Partial lung resection of the left S6 was carried out by videoassisted thoracoscopic surgery. The pathological diagnosis of the left S6 was epithelial granuloma with caseation. A culture of the same resected specimen was positive for Mycobacterium avium. The eventual clinical staging for the squamous cell carcinoma in the right S10 was cT2N0M0 (IB). Radical surgical treatment and right lower lobectomy were performed for the S10 lesion. We considered that 18F-FDG-PET was an effective noninvasive procedure for diagnosis of solitary or multiple solitary nodular shadows in the lung.

[Difficulties in recognition and treatment of lethal midline granuloma and otolaryngologic and radiotherapeutic practice]. / Uber die Schwierigkeiten bei der Erkennung und Behandlung des "Lethal midline granuloma" in der otolaryngologischen und strahlentherapeutischen Praxis.

The authors report on two cases of a "lethal midline granuloma". The first patient was treated by irradiation and chemotherapy. He lives for the last three and a half years in complete remission. The established diabetes did not affect the healing process. In the second case only a palliative irradiation was practicable because of the extreme tumour propagation, but the result was nevertheless good.

[Malignant granuloma of the mid-face: value of x-ray computed tomography/MRI imaging]. / Granulomes malins centro-faciaux: intérêt de l'imagerie TDM/IRM.

CT an MR imaging are now an integral part of the work-up of every ulcerative lesion of the mid-face. The CT scan enables an exhaustive evaluation of the bone and cartilage necrosis by using bone densitometry settings. The mass lesions, the inflammatory areas and the thickened mucosa are best demonstrated using the tissue densitometry settings. Injection of iodinated contrast material makes clearer delineations of the abnormalities, but doesn't differentiate well between muco-purulent fluid retentions (in the sinuses for example) and solid neoplastic masses. MRI is the technic of choice for this goal; it also gives helpful images in the sagittal and frontal planes. CT and MR imagings are best combined to guide the surgical biopsies and the radiation therapy when managing the difficult problem of mid-face ulcerative lesions.

CT evaluation of polymorphic reticulosis.

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.

A review of the role of radiology in non-healing granulomas of the nose and nasal sinuses.

This paper reviews the radiological features of 20 patients with Wegener's granulomatosis and seven cases of lethal midline granulomas. It compares the findings of plain sinus radiographs and tomography. There are no specific radiological features of either Wegener's granulomatosis or lethal midline granuloma. Plain sinus radiographs underestimate the extent of bony changes and the differences between Wegener's and lethal midline granulomas are one of degree. Computerised tomography will show the extent of the disease. The role of nuclear magnetic resonance is also discussed. Nuclear magnetic resonance does not show bone destruction but the T2 weighted response of the tissues helps to differentiate the condition from neoplasia. The literature is reviewed and compared with the study.

Computed tomography of lethal midline granuloma. 5 case reports.

Five cases of lethal midline granuloma are evaluated with computed tomography. CT examination is indispensable to appreciate extension of facial lesions, and detect lymphoma often associated.

Radiology of the paranasal sinuses in non-healing granulomas of the nose.

The few accounts of the radiographic features in the nose and sinuses of Wegener's granulomatosis and lethal midline granuloma have involved small numbers. This paper reviews these changes in 20 cases of Wegener's granulomatosis and 7 cases of lethal midline granuloma. Fifteen cases of Wegener's granulomatosis showed either negative or non-specific changes on plain sinus views. In the remaining 5 cases some evidence of bone destruction was seen. The range of changes was much greater in lethal midline granuloma. The radiographic changes varied from normal appearances (1 case) through generalized soft tissue opacity (3 cases), to massive destruction of the nasal bones and sinus walls (3 cases). The difference in radiological pattern in Wegener's and lethal midline granuloma would seem to be one of degree only.

Computerized tomographic evaluation of early idiopathic midline granuloma.

Idiopathic midline granuloma (IMG) is a rare, slowly progressive inflammatory process producing localized destruction of the nasal mucosa, septum, paranasal sinuses, and palate, often associated with erosion through central facial tissue and bone. We review some of the salient features of IMG and present the first reported CT demonstration of this disease.

Laryngeal tuberculosis.

Laryngeal lesions observed in patients with pulmonary tuberculosis are most likely tuberculous. Although tuberculosis and carcinoma may be indistinguishable clinically and radiographically, specific radiographic studies better delineate the lesion and may suggest its true etiology. Five cases of laryngeal tuberculosis referred to M.D. Anderson Hospital and Tumor Institute as carcinoma of the larynx are described and illustrated. In the acute exudative type, inflammatory disease was suspected, but in the chronic localized lesion, carcinoma was the primary consideration. The histologic diagnosis was established by biopsy.