Pigmented villonodular synovitis. A clinicopathologic study of 52 cases

Clinicopathologic, enzyme histochemical and electron microscopic findings in 52 patients with pigmented villonodular synovitis (PVS) are reported. The lesion was by far the most common in the knee joint (48%), followed by the ankle joint (25%). As to sex incidence, there seemed to be no predilection (46% in men, 54% in women). Microscopically, the PVS showed thin or thick villous projections of the involved synovial membrane, associated with or without nodular formation. The nodule of PVS consisted essentially of a proliferation of histiocyte-like cells with phagocytic activities. Another characteristic feature was large clefts and pseudoglandular or alveolar spaces lined by synovial cells. Enzyme histochemical studies revealed that the lesional cells had functional properties of macrophages. Electron microscopically, the lesion consisted essentially of histiocyte-like and fibroblast-like cells, together with intermediate cells and myofibroblasts.     

Liposarcoma of the breast: a clinicopathologic study of 20 cases

The clinical and pathologic features of 13 patients with pure liposarcomas of the breast and seven patients with liposarcomas that had arisen in cystosarcomas are reviewed. Metastases occurred in three of the 13 patients with pure mammary liposarcomas, and two women died of tumor. One of the patients with a liposarcoma that had arisen in a cystosarcoma had a local recurrence one year after diagnosis but was subsequently lost to follow-up study. Fourteen of the 20 patients for whom follow-up information was available for as long as 14 years had no evidence of recurrence. Features associated with the development of recurrence, based on the cases studied here and previous reports, included the pleomorphic liposarcoma pattern and an infiltrative margin. Features associated with tumor-free survival included the well-differentiated liposarcoma pattern, male gender (two patients), and a circumscribed microscopic tumor margin. There were no axillary lymph node metastases. Follow-up data indicate that complete surgical excision of tumor with tumor-free margins is necessary, but total mastectomy and removal of the axillary tail are not required unless these procedures are needed for complete excision.     

Liposarcoma. An ultrastructural study of 15 cases

Fifteen liposarcomas from 13 patients were examined by electron microscopy. These included nine primary tumors, four recurrent tumors after primary surgery or irradiation, and two metastatic lesions. Twelve of the liposarcomas were located in the thigh, and 11 were of the myxoid variety. All neoplasms were composed of cells having the ultrastructural characteristics of some stage of lipoblastic differentiation, i.e., lipid droplets, micropinocytotic vesicles, glycogen, external lamina, intermediate filaments, Golgi apparatuses, rough and smooth endoplasmic reticulum, and mitochondria. The nuclear-cytoplasmic ratio and nuclear pleomorphism were related inversely to the size and number of lipid droplets. Lipoblasts were frequently in close association with capillaries and pericytes, and in four cases lipid droplets were found in pericytes. Multivacuolated, mitochondria-rich lipoblasts, resembling brown fat cells, also were seen. Most tumors contained lipid-free, poorly differentiated mesenchymal cells that showed a continuum of morphologic differentiation to cells that closely resembled early lipoblasts that contained nonmembrane-bound lipid vacuoles. Fibrolipoblasts, cells containing lipid droplets and abundant rough endoplasmic reticulum, were observed only in well-differentiated liposarcomas. Some soft tissue sarcomas contain vacuolated cells that simulate lipoblasts by light microscopy but prove to be reactive or malignant fibroblasts, histiocytes, or smooth muscle cells ultrastructurally. Therefore, use of electron microscopy may be necessary to establish the line of differentiation in these neoplasms.     

Superficial leiomyomas. A clinicopathologic study of 34 cases

The clinicopathologic findings of 34 cases of cutaneous and subcutaneous leiomyomas were described. These were composed of 12 cases of piloleiomyoma and 22 of genital leiomyoma (scrotum, nipple and areola, vulva, and perianal region). Thirteen of 22 genital leiomyomas occurred in the vulva. Four cases of leiomyoma of pilar arrector origin had multiple lesions. The tumor occurred predominantly in adult females with an approximate sex ratio of 1:3 (8 males and 26 females). The patients with such leiomyomas ranged in age from 26 to 77 with an average of 46.8 years. The histologic feature in each case was essentially the same, and composed of interlacing bundles of smooth muscle cells, although vulvar leiomyomas frequently showed varying degrees of hyalinization and were composed of more slender tumor cells. All 12 cases submitted for immunohistochemistry showed a strongly positive staining both for actin and for desmin. In our view, cutaneous and subcutaneous leiomyomas, excluding angioleiomyomas, are undoubtedly rare in occurrence.     

Intraosseous lipoma. A clinicopathologic study of four cases.

We describe the clinical, radiologic, and pathologic features of four cases of intraosseous lipoma. The patients ranged in age from 37 to 48 years, and three of them were men. Three patients presented with pain; in the fourth patient, the lesion was discovered incidentally. The involved bones were distal radius (in one patient), humerus (in two patients), and calcaneus (in one patient). In all cases, plain films revealed well-defined lytic lesions. Computed tomographic scan in all cases, and magnetic resonance imaging in one case, showed the lesions to have attenuation values and signal intensity identical to adipose tissue. Histopathologic examination of the curetted lesions revealed mature adipose tissue devoid of hematopoietic elements. Dystrophic calcification was present in two cases, necrosis of fat in one case, and extensive recent and remote hemorrhage in another case. As the roentgenographic differential diagnosis can be extensive, familiarity with this entity is important. These cases emphasize the characteristics of intraosseous lipoma, and they highlight the need for clinicopathologic and roentgenographic correlations.     

Myositis ossificans. A clinicopathologic study of 21 cases

This clinicopathologic study concerns 21 patients with myositis ossificans, whose ages ranged from 7 to 81 years (average, 40.3 years). The lesions were located in the thigh (10 cases), upper arm (6 cases), and other sites (5 cases). In all patients the lesion was solitary, and in all but one, it was seen within the muscle. Histologically, the lesions exhibited a wide range of histologic features with different amounts of immature fibroblastic cells, osteoid, cartilage, and young or mature bone accompanied by fibrous connective tissue. They could be classified into three types according to the predominant or most striking histologic features. Type I (6 cases) was characterized by highly cellular areas with islands of osteoid, having occasionally been confused with extraskeletal osteogenic sarcoma. Type II lesions (8 cases) consisted mainly of osteoid and young bone rimmed by osteoblasts, in the occasional presence of cellular areas. Type III lesions (7 cases) were made up almost wholly of mature bone and cartilage surrounded invariably by dense fibrous connective tissue. Prognosis was excellent in 17 patients for whom follow-up information was available. Difference between Type I myositis ossificans and extraskeletal osteogenic sarcoma was briefly described, following an additional review of three cases of the latter.     

Q fever. A clinicopathologic study of five cases

Bone marrow and liver biopsy specimens from five patients with documented Q fever were reviewed. Eight bone marrow and two liver specimens had been obtained from eight days to two months after the onset of symptoms in the five patients. Three had Q-fever hepatitis; one had Q-fever endocarditis. The classic "doughnut" granulomas of Q fever were present in either the liver or initial bone marrow biopsy in all four of the untreated patients. It was recognition of the classic granulomas that prompted serologic studies for Q fever in three of the four patients.     

Autoimmune gastritis. A clinicopathologic study of 25 cases

The histopathological diagnosis of autoimmune gastritis (AG) in its early stages can be a diagnostic challenge. Even some advanced cases with complete atrophy of the corpus mucosa may be difficult to recognize. To establish the diagnosis of autoimmune gastritis, several histological features should be assessed and combined with immunostains for enterochromaffin cell-like (ECL) cells and G-cells. The main histological criteria include a mononuclear infiltrate within the lamina propria, foci of destruction of oxyntic glands, intestinal metaplasia (IM), pyloric metaplasia, and parietal cell pseudohypertrophy. These criteria were evaluated in our series of 25 patients with achlorhydria and/or megaloblastic anemia. Some of our patients presented with nonspecific gastrointestinal symptoms. The age ranged between 46 and 79 years; one male patient was only 31 years old. Histologically, the corpus mucosa displayed in all cases chronic inflammation with focal complete IM and advanced pyloric metaplasia. In 4 patients, oxyntic glands were destructed in some sites. There was a pancreatic metaplasia of acinar type in 2 patients and a minimal focal pseudohypertrophy of parietal cells in the 31-year-old man. A tubular adenoma with a low-grade dysplasia was found in one female patient. Immunohistochemically, chromogranin-A highlighted linear or nodular hyperplasia of ECL cells in 19 patients, and adenomatoid ECL hyperplasia in one case (80%). In the remaining cases hyperplasia of ECL cells could not be recognized from their normal count. In 13 cases (52%) a few ECL cells were seen also in IM. Regarding associated pathology, in one woman with nodular ECL cell hyperplasia, a gastric carcinoid was removed endoscopically. The reaction with gastrin antibody revealed in 11 cases (44%) a small number of G cells in IM in the corpus mucosa. In 18 patients, antral mucosa was examined as well. In 8 patients, the mucosa was normal; in 10 cases, a mild chronic inactive gastritis was diagnosed, and in 15 patients G-cell hyperplasia was found. In accordance with other studies, we show that the diagnosis of AG may be established microscopically in endoscopic specimens of the gastric body mucosa when histologic features and immunohistochemical detection of ECL and G cell hyperplasia are combined.     

Multilobated B-cell lymphoma. A clinicopathologic study of 24 cases

The clinical, histologic, and immunologic features of 24 cases of multilobated B-cell lymphoma are presented and compared with those of previously reported cases. Cases were included in this study when more than 50% of the tumor cells had nuclear multilobation. There were 13 males and 11 females with an age range of 13 to 79 years. A slight predominance of node-based presentations was observed. Seventeen patients (71%) presented with advanced (stage III or IV) disease. Complete remissions were achieved in 13 (59%) of 22 patients, and 14 (58%) of the 24 patients were still alive after a follow-up period ranging from 10 to 124 months. The 1-year survival was 65% (SE = 10%). Relapses occurred in 4 (31%) of 13 patients with complete responses. These observations, together with those previously reported, support the conclusion that the multilobated B-cell variant of non-Hodgkin's lymphoma behaves in a fashion similar to that of the intermediate-grade, diffuse, large-cell non-Hodgkin's lymphomas.     

Malignant fibrous histiocytoma. A clinicopathologic study of 130 cases

A clinicopathologic study of 130 cases of malignant fibrous histiocytoma (MFH) of the soft tissues is reported. This malignant neoplasm principally of middle and late adults occurred most often in the proximal portions of the extremities (48%) including the thigh and buttocks (35%). MFH may be subclassified into common (storiform and pleomorphic), myxoid, xanthogranulomatous, and giant cell types, the common type being accounted for 100 cases (77%) of the series. The prognosis was more favorable in patients with storiform and myxoid tumors than in patients with pleomorphic or other type tumors, the overall relative five-year survival rate being 48%. The depth of the tumor also affected prognosis with a significantly lower survival rate in deeply situated tumors. The rate of local recurrence of the tumor was 48%. Because of incomplete informations, metastasis was confirmed in only 26 patients and was most frequently to the lung (73%). In addition, electron microscopic, histochemical and tissue culture findings in limited cases are presented, concerning the histogenesis of the MFH.     

Cutaneous malignant lymphomas. A clinicopathologic study of thirty-seven cases

Clinical and histological findings in 37 cases of cutaneous lymphomas other than mycosis fungoides and Sezary syndrome were investigated; there were 31 in adults and 6 in children. Cutaneous lesions were the first manifestations of the diseases in all cases, and they appeared mostly as tumors or nodules. Cytomorphologically, about a half of the cases showed proliferations of large cleaved, non-cleaved cells or immunoblasts (Group I). Eight cases showed a polymorphous appearance containing convoluted cells of various size (Group II). Five cases in children demonstrated monomorphous proliferation of uniform-sized lymphoblasts (Group III). The cytologic findings in 6 cases did not fit into any lymphoid groups (Group IV). The clinical findings observed in each group were reviewed and compared. Follow-up study revealed that the prognosis of Group I was the poorest among the four groups.     

Dupuytren type fibromatoses. A clinicopathologic study of 62 cases

This clinicopathologic study concerns 62 patients with one of the Dupuytren type fibromatoses. Of the 62 patients, 42 had the lesion in the palm (palmar fibromatosis), 12 in the sole (plantar fibromatosis), 2 in the penis (penile fibromatosis), and 6 in the ectopic locations, the dorsal aspect of the hand in 3 patients, the dorsum of the fingers in 2, and the forearm in 1. The clinically palpable nodules were histologically classified into those of the following three phases, according to LUCK'S classification: proliferative phase (27), involutional phase (23), and residual phase (12). In addition to fibroblastic proliferation, the lesion of the proliferative phase exhibited vascular features consisting of one or more layers of cells with oval nuclei around the vessels within the nodule. In the lesions of the late proliferative and involutional phase, the intracytoplasmic fine filaments were visible in cells compatible with myofibroblasts by Masson's trichrome stain. The nuclei of the elemental fibroblasts in the same phases were often crossed transversely by one or more fine basophilic lines.     

Liposarcoma of the pleura: an exceptional tumor. A study of two cases

Primitive intrathoracic liposarcomas are rare tumors mostly located in the mediastinum or in lung parenchyma. Primitive liposarcomas of the pleura are exceptional and show a dramatic clinical course. These tumors are treated by surgery sometimes associated with radiotherapy. We report 2 cases of primitive liposarcoma of the pleura occurring in 2 women of 19 and 62 years of age. Histological examination showed in both cases a low-grade myxoid liposarcoma. In one case, ultrastructural study confirmed the diagnosis.     

Apocrine mammary carcinoma. A clinicopathologic study of 72 cases

Apocrine carcinoma (AC) is an uncommon, poorly characterized type of breast tumor. In this review, 55 patients with intraductal (ID) AC and 17 patients with infiltrating (IF) AC were analyzed retrospectively to define the histologic features and clinical course of this neoplasm. Recurrences in the breast occurred in 3 of 20 ID-AC patients treated by biopsy alone, but not in the 2 patients who received local radiation therapy after biopsy. One patient with ID-AC had axillary metastases at the time of treatment by mastectomy and died of disease five years later. The remaining patients with ID-AC treated by mastectomy have remained disease free. One of the three patients with IF-AC treated by biopsy alone died of disease, and one of two patients with IF-AC treated by biopsy and radiotherapy was alive with carcinoma. Twelve patients with IF-AC were treated by mastectomy. Ten of them were recurrence free at the time of last observation. More than one-third of the cases of ID-AC and IF-AC were detected by mammography alone. Survival analysis of IF-AC cases compared with nonapocrine duct carcinoma cases matched for stage revealed no statistical difference in estimated recurrence-free survival or estimated survival probability. AC is a distinct morphologic entity with a natural history similar to that of nonapocrine ductal carcinoma.     

Desmoplastic malignant melanoma. A clinicopathologic study of 14 cases

Clinical and pathologic details in 14 cases of desmoplastic malignant melanoma were reviewed. The study group included ten men and four women with a median age of 58 years. Anatomic locations such as the head and neck area (four cases), limbs (five cases), and trunk (five cases) were involved with equal frequency. Follow-up information (median period, 4.6 years) was available for 12 patients, of whom four are alive and disease free, six have had local tumor recurrence, and two have died of their disease. Histologically, these lesions consisted of a malignant fibroblastic skin tumor intimately associated with a superficial melanoma (ten cases) or melanocytic dysplasia (four cases) that often extended deeply to the subcutaneous fat. Helpful diagnostic features included the presence of neurotropism, a lymphocytic infiltrate, and unusual patterns of triangular and periadnexal lamellar fibroplasia. Of the immunohistochemical markers employed, antisera to S100 protein and vimentin yielded the most consistent positive results. Immunostaining with NK1/C-3 (antimelanoma monoclonal antibody) was not helpful. Ultrastructural evidence of fibroblastic and schwannian differentiation was seen. We conclude that the altered morphologic melanomas is associated with a relatively favorable prognosis and believe that careful attention to light microscopic detail with immunohistochemical and electron microscopic assistance will elucidate the diagnosis in most cases.     

Immunohistochemistry-based subtyping of breast carcinoma in Egyptian women: A clinicopathologic study on 125 patients

Breast carcinoma is a heterogeneous disease affected by patients' ethnicity. Gene expression analysis identified several molecular subtypes, and similar subtyping has now been found to be feasible using immunohistochemistry. This study estimated the distribution of intrinsic breast cancer subtypes using estrogen receptor, progesterone receptor, human epidermal growth factor receptor 2 (Her2/neu), and cytokeratin 5/6 immunostaining in a cohort of 125 Egyptian women diagnosed as having invasive breast carcinoma. Associations with clinicopathologic variables and the prognostic markers Bcl-2 and Cyclin D1 were investigated and statistically analyzed. Population difference in breast cancer subtypes was detected, suggesting etiologic and genetic heterogeneity among demographic groups. As reported worldwide, most tumors were luminal A (39.2%), but basal-like and unclassified subtypes had higher proportions among our cohort (16.8% and 16%, respectively), particularly in premenopausal patients (P = .0001), in contrast to postmenopausal African Americans, premenopausal European Americans, and other populations. Her2-overexpressing subtype was the least common subtype (13.65%) among our patients, although it is more common in Asians. Basal-like and unclassified carcinomas were more frequently grade 3 neoplasms (P = .035). Lobular histology was distributed among luminal A, B and unclassified subtypes (P = .006). The highest frequency of nodal positivity was associated with Her2 overexpressing carcinomas (94.1%, P = .0001). Luminal and unclassified carcinomas more likely expressed Bcl-2 (P = .011) and Cyclin D1 (P = .0001), whereas basal and Her2 subtypes had the lowest expression levels. Immunohistochemistry-based subtyping can be helpful in separating breast carcinoma into subtypes that vary in distribution among different populations. These subtypes have distinct clinicopathologic features and diverse prognostication, which may imply different therapeutic options for each subtype.     

Hemangiomas of the heart. A clinicopathologic study of ten cases

The clinical and histopathologic features of 10 cardiac hemangiomas are presented. The patients' ages ranged from 2 weeks to 65 years (mean: 32 years). Eight patients had no other cardiovascular abnormalities, one was associated with intra-atrial lipoma, and one was associated with an anomalous origin of the left coronary artery. One patient had other hemangiomas of the oral cavity and small intestine. The tumors presented as pericardial effusion (2), sudden death (2), shortness of breath (1), incidental finding on chest X-ray (1), asymptomatic murmur (1), syncope (1), and incidental finding at autopsy (2). Location of the tumors included the right ventricle (4), left ventricle (3), atrial septum (2), and right atrium (1). All six tumors presenting in life were resected successfully, one requiring placement of a synthetic graft. Cardiac hemangiomas are usually sporadic; presenting symptoms depend on location in the heart; arrhythmias or sudden death can occur if the tumor is present in the basal region of the ventricular septum; surgical resection is feasible in other locations.     

The prognostic implication of thymoma histologic subtyping. A study of 80 consecutive cases

In this study the authors have investigated the clinicopathologic correlations in 80 consecutive cases of thymoma in order to establish the clinical usefulness of histologic subtyping of these tumours. All cases were histologically examined and classified according to Salyer and Eggleston and to Marino and Müller-Hermelink classifications. Therefore, thymomas were subtyped as predominantly lymphocytic, mixed and predominantly epithelial and cortical, mixed and medullary, respectively. The frequency of the different histologic subtypes was determined, and histologic findings were related to patients' age, surgical stage, and survival. Through the application of Salyer and Eggleston classification, the three histologic subtypes did not correlate with patients' ages at time of diagnosis, surgical stage as determined by local infiltration, and prognosis as determined by survival curves. On the contrary, when Marino and Müller-Hermelink classification was applied, statistically significant relationships between histologic results and age, surgical stage, and prognosis were demonstrated. These results and their implications are discussed, with special reference to the important problem of histogenesis of thymomas and of their clinicopathologic staging.