Biopsy findings in malignant histiocytosis presenting as lethal midline granuloma.

Nasal biopsy findings in malignant histiocytosis presenting clinically as lethal midline granuloma are characterised by necrosis and infiltration of atypical histiocytic cells with a diffuse positive reaction for non-specific esterase. This cellular character was common to midline malignant reticulosis, and midline malignant reticulosis and malignant histiocytosis are thought to be the same disease. Patterns of histiocytic infiltration in the nasal lesions of 19 cases are reported in this paper. Polymorphic and monomorphic patterns were observed in 11 and four cases respectively, on the initial biopsy, but subsequently the infiltrates frequently became monomorphic on serial biopsy. The reverse was not observed. Surface marker and cytochemical studies showed the true histiocytic nature of the proliferating cells, and necropsy findings justified the diagnosis of malignant histiocytosis.     

Peripheral T cell lymphoma presenting primarily as lethal midline granuloma

This clinicopathologic study reports seven patients who primarily presented with ulcerative and destructive lesions of the upper aerodigestive tract and face, clinically consistent with so-called lethal midline granuloma (LMG). Histologically, the infiltrates were composed of atypical lymphoid cells that displayed angiocentricity and angiodestruction. In five patients, involvement of distant sites such as skin, lungs, lymph nodes, and bone marrow occurred, and in two cases, the disease remained localized. Immunomorphologic analysis, using monoclonal antibodies to frozen and paraffin sections, provided evidence for the diagnosis of peripheral T cell lymphoma (PTL) in all cases. The midline tumors were classified as diffuse mixed or diffuse large cell lymphoma occurred at distant sites. According to modern PTL classification systems, the lesions could be classified as pleomorphic T cell lymphomas. Those five patients who presented with or progressed to large cell lymphoma died within 18 months (mean, 7 months), whereas the two patients with localized disease are alive after 10 and 36 months, respectively. The size of the atypical lymphoid cells may be of prognostic significance since the large cell compartment seems to represent the major growth fraction in these PTLs.     

Heterogeneous malignant non Hodgkin's lymphomas as a causative disorder in lethal midline granuloma

Summary The present report describes the results of a combined morphological, enzyme- and immunohistochemical analysis of nine cases of malignant non Hodgkin's lymphomas (NHL) clinically presenting as lethal midline granuloma. In a previous report written before antibodies directed against B and T lymphocytes were available, a histiocytic origin of such neoplasms had been suggested. A panel of antibodies reactive with most B cells (L26, MB1, KiB3) and a majority of T cells (MT1, UCHL1) was applied on paraffin sections of formalin fixed tissues as well as antibodies directed against leukocyte common antigen (LCA), myeloid/histiocyte antigen (MAC 387), lysozyme, alpha-1-antitrypsin, alpha-1-antichymotrypsin, S-100 protein, prekeratin and immunoglobulin light chains. Enzyme histochemistry included tests for non-specific acid esterase, acid phosphatase, betaglucuronidase and chloroacetate esterase. As a result, five T, two B and two unclassified (malignant histiocytosis probable) NHL were identified, indicating distinct heterogeneity of NHL as causative disorders in lethal midline granuloma.     

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.     

Polymorphic reticulosis (lethal midline granuloma) and lymphomatoid granulomatosis: Identical or distinct entities?

Summary Two cases of polymorphic reticulosis were studied. Both cases had a fatal clinical course, that of the second case being rapid and progressive and ending 6 months after the onset of the disease with little demonstrable effect of steroid therapy. Biopsy material was obtained in both patients, and both were submitted to a post-mortem examination. The first case showed typical angiocentric, angiodestructive, polymorphous lymphoreticular infiltrates, involving the pharyngeal region and the tongue. The second case demonstrated these same lesions in the midfacial region, the lungs and the skin. A possible identity between polymorphic reticulosis and lymphomatoid granulomatosis is discussed (because of the coexistance of identical lesions in the midfacial region and in the lung parenchyma in the second case). Wegener's granulomatosis in limited and disseminated forms and malignant lymphoma are considered in the differential diagnosis.     

Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture

Malignant histiocytosis is a rare invasive proliferation of neoplastic histiocytes. Cases previously reported as malignant histiocytosis were shown to be lymphomas of T or B lineage, especially anaplastic large-cell lymphomas. A case of malignant histiocytosis is described, in which a patient presenting with symptoms suggestive of pneumonia suddenly deteriorated and died. At autopsy, a large quantity of fresh blood, originating from several ruptured nodules on the enlarged spleen, was seen in the peritoneal cavity. Extensive infiltration by pleomorphic tumour cells and erythrophagocytosis by tumour cells were seen on histological examination of the spleen. Immunohistochemical analysis and staining were carried out. This is the second reported case of malignant histiocytosis presenting with spontaneous splenic rupture.     

Langerhans cell histiocytosis presenting as a thyroid gland mass

We report 2 cases of Langerhans cell histiocytosis (LCH) presenting as a thyroid mass. The first case is a 45-year-old woman with a 13-year history of diabetes insipidus who presented with an enlarging thyroid mass with substernal extension. The second case is a 29-year-old man who presented with an enlarging thyroid mass and skin lesions. Histologic evaluation of the thyroid gland in both cases revealed extensive involvement by LCH, confirmed by immunohistochemical analysis showing Langerhans cells that were positive for CD1a and S-100 protein. Langerhans cell histiocytosis can rarely involve the thyroid gland in adults, and we have identified 30 cases reported in literature. Most patients had evidence of LCH involving other anatomic sites, as was true in these 2 cases, and the diagnosis was initially established by examination of other sites in a subset of patients. Affected patients frequently have diabetes insipidus, as was true in case 1. Thyroid gland involvement as the initial presentation of LCH is a rare phenomenon that can result in misdiagnosis.     

Malignant histiocytosis in childhood: Morphologic Considerations

Eight cases diagnosed over a ten-year period as malignant histiocytosis (MH; histiocytic medullary reticulosis) were reviewed to clarify diagnostic criteria for the childhood disease and to identify sources of diagnostic confusion. Five of the eight cases met the authors' criteria for diagnosis; i.e., they were characterized by loose mixed infiltrates composed of three cell types--well-differentiated histiocytes, prohistiocytes, and malignant histiocytes--and they had no leukemic phase. Three cases did not share these features and were reclassified. The liver was found to be the organ most useful in premortem diagnosis, and immunoperoxidase staining for immunoglobulins and lysozyme was also helpful. The clinical and morphologic features of the five cases confirm the authors' view that diagnoses of MH should be limited to cases in which there is a loose pleomorphic population of all three types of histiocytes and that cases with monomorphous populations of aggregated malignant cells should be classified as lymphomas.     

Malignant histiocytosis: a study on clinicopathological features and cell origin]

Thirty-one autopsy cases previously diagnosed as malignant histiocytosis (MH) were studied by means of immunohistochemical staining. Antibodies detecting the formalin resistant epitopes on T-cells, B-cells and those of histiocyte/monocyte origin were used. It was shown that the malignant histiocytes reacted only to the cell markers derived from histiocyte/monocyte, and only a part of lymphocytes showed positive reaction to the T and B cell markers. It is suggested that the histiocyte/monocyte lineage is the possible origin of the malignant proliferating cells in MH. The clinicopathological features and the differentiation of MH from familial erythrophagocytic lymphohistiocytosis, virus-associated hemophagocytic syndrome and malignant lymphoma are described. The pathogenesis, the causes of death and the points for attention in the treatment of MH are also discussed.     

Malignant schwannomas presenting as malignant fibrous histiocytomas

Two soft tissue neoplasms considered to represent malignant fibrous histiocytomas by light microscopy showed typical findings indicative of Schwann cell origin when examined by electron microscopy. These findings included the presence of cells exhibiting long interdigitating cytoplasmic processes, which frequently contained collections of microtubules and were surrounded by thin, frequently interrupted, basal lamina material. In addition, pinocytotic activity at the cell surfaces and immature junctions joining apposing cellular membranes were identified. No evidence of fibroblastic, fibrohistiocytic, or myofibroblastic differentiation was identified ultrastructurally in the neoplastic cells. Our findings stress the need for ultrastructural examination to adequately classify soft tissue sarcomas. The 2 cases presented illustrate that there are some schwannomas that may be inaccurately classified as malignant fibrous histiocytomas if only light microscopy is used. It seems that only by ultrastructural means is it possible to accurately classify these peculiar neoplasms.     

Malignant sarcomatoid mesothelioma of the pleura: a histological and immunohistological study of a case

The authors describe the case of a 70 year-old asymptomatic female, who showed nodular pleural growths at an occasional chest roentgenogram. Histological examinations and laboratory findings excluded the possibility of an inflammatory granulomatous process, but they did not allow differentiation between malignant sarcomatoid pleural mesothelioma and malignant fibrous histiocytoma. Only at immunohistochemistry was the differential diagnosis made. In fact, the co-expression of vimentin and cytokeratins by neoplastic cells, along with their negativity for macrophage markers strongly supported the hypothesis that the neoplastic growth originated from subpleural mesenchymal stem cells.     

Congenital histiocytosis. A heterogeneous group of diseases, one presenting as so-called congenital self-healing histiocytosis

Three cases of congenital histiocytic disorders--generalized Langerhans cell histiocytosis, generalized juvenile xanthogranuloma and so-called congenital self-healing histiocytosis are compared using histiochemical, immunohistochemical and ultrastructural methods. The results showed a typical morphological pattern of Langerhans cell histiocytosis (S 100+, CD 1+, alpha-mannosidase +) with an unusual self-healing cutaneous phenomenon. The congenital self-healing histiocytosis showed a non-Langerhans cell immunophenotype (CD 14+, CD 1-, S 100-) and morphological appearance resembling the evolutive "early" stage of juvenile xanthogranuloma. A diffuse cellular positivity of alpha-mannosidase in juvenile xanthogranuloma and congenital self-healing histiocytosis differed from a typical perinuclear globular positivity of this enzyme in Langerhans cell histiocytosis. It is concluded that congenital self-healing histiocytosis may in some cases be of non-Langerhans cell type and under this term a clinically characteristic syndrome of histiocytic proliferation of Langerhans cells or tissue histiocytes may be included.     

Angioleiomyomas: an immunohistological study

In a series of nine angioleiomyorlias, nerves were identified immunohistologically within the capsule and interstitium only in those that were painful. We believe that it is stretching of capsular nerves that underlies most of the patients symptoms.     

Malignant histiocytic tumour presenting as a primary uterine neoplasms: a cytochemical and electron microscopy study

A 41-year-old woman was admitted because of uterine bleeding. The diagnosis of malignant histiocytic tumor was suspected from microscopic examination of the material obtained with diagnostic curettage. Total hysterectomy was performed a week later and a complete pathological examination of the specimens was performed. Cytochemical and immunoperoxidase stains and electron microscopy confirmed that the neoplastic cells were derived from histiocytic elements, i.e. from the mononuclear phagocyte system.     

Malignant histiocytosis in children (pathologicoanatomic study of 16 cases)

Malignant histiocytosis is a rare neoplastic disease from the group of acute leukemias. Morphological characteristics of the disease are presented on the basis of histological, histochemical, and electron microscopic examinations of section materials from 16 cases of malignant histiocytosis in children of 6 to 14 years. Systemic focal-diffuse proliferation of tumor cells of histiocytic type of various degrees of differentiation was observed in organs and tissues. Most frequently, typical macro- and/or microscopic lesions were detected in lymph nodes, bone marrow of vertebrae, flat and tubular bones, liver, and spleen. The histiocytic nature of tumor cells was judged primarily from such signs as their capacity for phagocytosis detectable by light and electron microscopy, high diffuse cytoplasmic activity on nonspecific esterase and acid phosphatase, and features of the ultrastructure. In the establishment of the diagnosis, malignant histiocytosis had to be differentiated from Letters-Siwe disease, sinus histiocytosis with massive lymphadenopathy, lymphogranulomatosis, immunoblastic lymphosarcoma, melanoma, undifferentiated cancer.     

Malignant histiocytosis. Clinicopathologic review of 18 cases with immunohistochemical study

We reviewed clinical and pathologic characteristics of 18 cases of malignant histiocytosis including 2 autopsy cases with special interest on their immunohistochemical characteristics. We report 3 cases of unusual immunohistochemical finding and postulate these cases may be a supportive evidence of the view that dendritic cells share common precursor with those of mononuclear phagocytic system. More accumulated cases in the future and careful analysis would be needed to answer the basic question about the origin of malignant histiocytosis.