Liselotte Hochholzer

Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases.

We reviewed 223 localized fibrous tumors of the pleura and divided them histologically into 141 benign and 82 malignant neoplasms. The criteria used for a judgment of malignancy were high cellularity and mitotic activity (more than four mitotic figures per 10 high-power fields), pleomorphism, hemorrhage, and necrosis. The tumors occurred equally in both sexes, most commonly in the sixth to seventh decades of life. Presenting symptoms included chest pain, dyspnea, and cough; they were observed in three-fourths of patients with a malignant tumor. One in every four of these patients had hypoglycemia, clubbed digits, or pleural effusion. Two-thirds of the tumors were attached to visceral pleura, often by a pedicle. The rest arose from the parietal pleura of the chest wall, diaphragm, or mediastinum. Neoplasms in these atypical sites, together with fissural lesions and tumors “inverted” into peripheral lung, were more often malignant. Most neoplasms measured 5–10 cm and weighed 100–400 g. Microscopically, the “patternless pattern,” or hemangiopericytic type, was seen in the majority of cases, and mixed patterns were seen in nearly 40% of tumors. Of the 169 tumors where follow-up was available, all of the benign and 45% of the malignant tumors were cured by simple excision. Patients surgically cured of a malignant neoplasm had pedunculated or well-circumscribed lesions. However, 55% of patients with malignant tumors succumbed to their disease secondary to invasion, recurrence, or metastasis. Resectability is the single most important indicator of clinical outcome. No tumor expressed epithelial differentiation, either immunohistochemically or ultrastructurally; therefore, we favor the term “localized fibrous tumor” of pleura instead of “localized mesothelioma.”

Mucoepidermoid tumors of the lung

Mucoepidermoid tumors of lung (MET) are rare tumors derived from the minor salivary gland tissue of the proximal tracheobronchial tree. The authors studied 58 cases of MET confined to the lung and used criteria derived from similar tumors of the salivary glands to separate them into low‐grade and high‐grade variants. The overwhelming majority of low‐grade tumors behaved in a benign fashion, whereas 23% of high‐grade tumors resulted in patient death. Prognostic factors which appeared to predict future aggressive behavior included high‐grade classification, advanced stage at presentation, and perhaps lymph node metastases.

Lymphoid interstitial pneumonia: clinicopathological and immunopathological findings in 18 cases

&NA; Eighteen patients with lymphoid interstitial pneumonia (LIP) were studied. The diagnosis was established by the microscopic finding of interstitial infiltrates of lymphocytes and plasma cells. Forty‐seven% of patients also had germinal centres, while 72% showed interstitial giant cells. Cases studied by the immunoperoxidase technique showed the interstitial plasma cells to be polytypic. The median age of patients was 56 years; most had cough, dyspnea, or chest pain. Chest X‐rays showed either patchy interstitial infiltrates (usually bilateral) or poorly defined nodules. Ten patients had hypergammaglobulinemia; one had hypogammaglobulinemia. Two patients had Sjögrens syndrome, two had biopsy‐proven chronic active hepatitis, and two had a clinical diagnosis of primary biliary cirrhosis. Follow‐up examination of 14 patients showed clearing of symptoms, X‐ray infiltrates or stable infiltrates in 4 cases each. Five patients died (mean survival, 41 months), one of whom succumbed to disseminated lymphoma and a second to respiratory failure. Our results support the hypothesis that LIP is a non‐neoplastic cellular proliferation in which lymphoma may supervene. The high incidence (22%) of chronic liver disease has not previously been noted.

Ganglioneuroblastoma of the posterior mediastinum: A clinicopathologic review of 80 cases

The pathology and clinical course of 80 cases of ganlioneuroblastoma (GNB) of the posterior mediastinum are presented. Many neoplasms in this series had previously been classified as differentiating neuroblastomas or immature ganglioneuromas. The prognosis for this group is far better than that for either neuroblastomas or intra‐abdominal GNBs. Follow‐up was obtained for 72 patients, of whom 55 were followed for 2–23 years. The five‐year actuarial survival rate was 88%. Prognosis was related to histologic growth pattern, age, and extent of disease at diagnosis. Stage I tumors were curable by simple excision; children with Stage II tumors treated with surgery and radiation responded as favorably as did those who received adjunctive chemothrapy. In contrast to those associated with late recurrences, the only examples of fatal primary tumors, with one exception, were those patients with Stage IV lesions at the time of initial diagnosis. The therapeutic implications and need for uniform terminology in classifying GNBs are discussed.

Lymphomatoid granulomatosis: A clinicopathologic study of 42 patients

&NA; We studied the histological and clinicopathological findings in 42 patients who had lymphomatoid granulomatosis (LYG). In addition to small round lymphocytes, small to intermediate lymphocytes with serpentine nuclei, large immature mononuclear lymphoid cells, abundant histiocytes, and vascular invasion by the cell infiltrate were observed in all cases. Fifty percent of lesions had occasional “atypical” cells with multi‐lobed nuclei. Three of four follow‐up autopsies showed large cell lymphoma, while one other autopsy and the single repeat biopsy showed increased numbers of large immature mononuclear lymphoid cells. Patients were most frequently men 40–60 yr old who had a history of pulmonary symptoms, such as cough or chest pain, and who showed multiple bilateral lung nodules without hilar adenopathy in the chest x‐ray. Thirteen patients (38%) died of disease, 11 of them within 12 mth of initial diagnosis. The presence of neurological signs and symptoms, increased mitoses, or increased numbers of atypical multi‐nucleated cells in the initial biopsy were not statistically significant predictors of survival.

Malignant fibrous histiocytoma of the lung

Twenty‐two cases of primary malignant fibrous histiocytoma (MFH) of the lung are described, and a review of the literature is presented. As in the soft tissue, this sarcoma is found primarily in older individuals, usually as a solitary peripheral mass. Chest wall invasion at diagnosis was seen in four cases. The most common histologic subtype was the storiform‐pleomorphic type of MFH. Vascular invasion was seen in 50% of cases. Histologic features did not correlate with prognosis, and subsequent aggressive behavior was related primarily to symptomatic presentation, advanced stage at diagnosis, incomplete excision, invasion of the chest wall or mediastinum, and subsequent recurrence or metastasis. Primary MFH of the lung should be distinguished from spindle cell carcinoma and inflammatory pseudotumors, and this histologic differential diagnosis is discussed.

Allergic granulomatosis (Churg-Strauss syndrome): Pulmonary and renal morphologic findings

The pulmonary histopathology of four cases of allergic granulomatosis (Churg-Strauss syndrome) was reviewed. Renal tissue was also studied in one case. The patients were young and most presented with asthmatic symptomatology. They showed marked peripheral blood eosinophilia, and had fluffy nodular pulmonary infiltrates by chest x-ray. Serum IgE was elevated in the one patient in whom it was obtained. The lung tissue in all cases showed necrotizing giant-cell vasculitis, interstitial and perivascular granulomas, and eosinophilic pneumonia-like areas. These microscopic features distinguish allergic granulomatosis from other forms of pulmonary eosinophilia or vasculitis. Renal tissue showed necrotizing granulomatous vasculitis and interstitial eosinophilic nephritis, without evidence of glomerulonephritis. Electron-microscopic study of one lung biopsy and of the renal tissue demonstrated tissue eosinophilia and, in lung, a granuloma. There was no evidence of vascular or glomerular electron-dense deposits. These findings are discussed in the light of possible pathogenetic mechanisms of allergic granulomatosis.

Truly Benign “bronchial Adenoma” Report of 10 Cases of Mucous Gland Adenoma with Immunohistochemical and Ultrastructural Findings

Mucous gland adenoma of the bronchus (MGAB) is a rare, solitary, benign, well-circumscribed, multicystic, predominately exophytic bronchial tumor. Mucous gland adenoma arises from the submucosal seromucous glands and ducts of proximal airways; since the location, signs and symptoms, and bronchoscopic findings are similar to those of other tumors arising in the bronchus, diagnosis depends on tissue biopsy. We herein report 10 patients with MGAB. Patients ranged in age from 25 to 67 years old (mean, 52). In two thirds of patients, the tumor was located in the middle or lower lobes. Tumors ranged in size from 0.8 to 6.8 cm (mean, 1.8). Cut surface was shiny, mucoid, cystic, and usually firm. Mucous gland adenomas are protean in their histologic patterns. They may appear glandular and tubulocystic or papillocystic, and they often show a mixture of these features. The tumors are rich in mucins and are immunopositive for epithelial markers. Mucous gland adenoma needs to be distinguished from low-grade malignant tumors of the bronchus—most notably, low-grade mucoepidermoid carcinoma. Complete removal of the tumor is curative.

Primary pulmonary hemangiopericytoma

Eighteen cases of primary pulmonary hemangiopericytoma are reported. These pulmonary sarcomas tend to occur in older individuals, frequently as asymptomatic, non‐calcified solitary masses on chest radiographs. Morphologically, they have a histology similar to their soft tissue counterparts. No single clinical or histologic feature allowed prediction of biologic aggressiveness in these are rare pulmonary sarcomas and the authors recommend that all hemangiopericytomas of lung be considered as potentially malignant.

Primary intrapulmonary meningiomas : A clinicopathologic and immunohistochemical study of ten cases

Primary intrapulmonary meningiomas are rare, and their occurrence has been reported in the literature only sporadically.