Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients

Epithelioid smooth-muscle tumors of the uterus are uncommon neoplasms for which prognostic factors have not been well established. A retrospective follow-up study of 18 uterine epithelioid smooth-muscle tumors was performed. Patients ranged in age from 27 to 83 years (mean, 45 years) and were separated into three groups based on the nuclear grade of the epithelioid tumor cells. Two tumors had grade 1 nuclei; both were examples of intravenous leiomyomatosis. They had highest mitosis counts of 1 and 3 mitotic figures (MF)/10 high-power fields (HPF), no tumor cell necrosis was found, and both patients were alive with no evidence of disease at 64 and 5 months' follow-up. Ten tumors had grade 2 nuclei. All had highest mitosis counts of 0 to 3 MF/10 HPF, except one (5 MF/10 HPF). Tumor cell necrosis was absent in nine and only one had an infiltrative border. Tumor size ranged from 1.5 to 14 cm. Two tumors contained pleomorphic ("symplastic") multinucleated giant cells similar to those seen in bizarre leiomyomas. All nine patients with follow-up were alive with no evidence of disease 5 to 203 months postoperatively (median, 74 months). One patient had also received adjuvant radiation therapy. Six tumors had grade 3 nuclei. Highest mitosis counts of 4 to 9 MF/10 HPF were found in five; one had 1 MF/10 HPF. Maximum tumor size ranged from 4.5 to 13 cm. Two had tumor cell necrosis, and two had an infiltrative border. Two of these patients died of tumor 11 and 132 months postoperatively. The former had widespread metastases at initial surgery (stage IVb); the latter patient (stage I) developed the first of seven tumor recurrences 3 years postoperatively. Both patients had also received adjuvant chemotherapy. Of the remaining four patients, two were alive with no evidence of disease at 48 and 83 months, one was alive (tumor status unknown) at 28 months, and one was lost to follow-up. Based on our findings and those in the literature, we conclude that uterine smooth-muscle tumors with a predominance of epithelioid cells are extremely uncommon and metastasize infrequently. No single histologic feature is predictive of metastatic potential. Clinically malignant tumors (i.e., epithelioid leiomyosarcomas) typically have the combination of significant nuclear atypia (either grade 2 or grade 3 nuclei) and some mitotic activity (usually at least 3 to 4 MF/10 HPF); most also have tumor cell necrosis.     

Leiomyosarcoma of the small intestine and the colorectum. A case contribution

Smooth muscle tumors of the alimentary tract are uncommon. A retrospective study was made of 7 patients treated for leiomyosarcoma of the small and large bowel to identify prognostic factors that influence results. The symptoms associated with these tumors varied according to the anatomic sites of the lesions and the position of the growth in relation to the intestinal lumen but the most common presenting clinical signs are bleeding or obstruction. The differential diagnosis between benign and malignant smooth muscle tumors is sometimes quite difficult. Clinical behavior of the myosarcomas of the gastrointestinal tract can be predicted to a large extent by the site of the tumor, the presence or absence of invasion of adjacent vital organs, and the histopathologic grade of malignancy. Although the mitotic activity of a gastrointestinal stromal tumor remains the most critical prognostic factor, tumors have been seen to recur locally and to metastasize even with rare or absent mitotic figures. Further studies are needed to pinpoint the factors that may be correlated to the prognosis.     

Sarcomas and related proliferative lesions of specialized prostatic stroma: a clinicopathologic study of 22 cases

Sarcomas and related proliferative lesions of the specialized prostatic stroma have been the subject of case reports and, thus, have not been well characterized. We reviewed the clinicopathologic features of 22 cases and studied the immunohistochemical profile of 9. Patient age ranged from 25 to 86 years; mean age was 54 years, and peak incidence was in the 6th and 7th decades. The most common clinical presentation was urinary retention, then abnormal results of digital rectal examination, hematuria or hematospermia, and a palpable rectal mass. The cases were grouped into two categories: prostatic stromal proliferation of uncertain malignant potential (PSPUMP, 18 cases) and prostatic stromal sarcoma (PSS, 4 cases) based on the degree of stromal cellularity and the presence of mitotic figures, necrosis, and stromal overgrowth. Four histologic patterns of PSPUMP were identified: (1) hypercellular stroma with scattered cytologically atypical cells associated with benign glands, (2) hypercellular stroma with minimal cytological atypia associated with benign glands, (3) hypercellular stroma with or without cytologically atypical cells, associated with benign glands in a "leaflike" growth pattern that resembled phyllodes tumors of the mammary gland, and (4) hypercellular stroma without cytologically atypical stromal cells and without glands. Prostatic stromal sarcoma showed greater cellularity, mitoses, necrosis, and stromal overgrowth than PSPUMP and consisted either of stromal elements with benign glands in a pattern that resembled malignant phyllodes tumors of the mammary gland (3 cases) or of purely stromal elements (1 case). Positive immunohistochemical reactions were noted using vimentin in 9 of 9 cases, CD34 in 8 of 8, HHF-35 in 2 of 8, smooth muscle actin in 3 of 9, desmin in 4 of 8, S-100 protein in 0 of 9, estrogen receptor in 1 of 7, and progesterone receptor in 6 of 7. None of the cases classified as PSS were positive for HHF-35, smooth muscle actin, or desmin. Of the 13 patients classified as having PSPUMP who did not undergo definitive local therapy at the time of diagnosis, recurrent signs or symptoms were seen in six (46%), necessitating additional therapy. Distant metastases to lung and bone developed in one patient classified as having PSS. Clinical and pathologic findings in this patient suggested a progression from PSPUMP to PSS. We conclude that sarcomas and related proliferative lesions of the specialized prostatic stroma encompass a spectrum of histologic features and may be grouped into two clinicopathologic categories: PSPUMP and PSS. Based on their distinctive histologic appearance and immunohistochemical profile, PSPUMP and PSS can be differentiated from other mesenchymal lesions of the prostate.     

Gastrointestinal autonomic nerve tumors. A clinicopathological, immunohistochemical, and ultrastructural study of 12 cases

The gastrointestinal autonomic nerve tumor (GAN tumor) is an uncommon stromal tumor of the intestinal tract and retroperitoneum first described by Herrera and associates in 1984. Distinction of GAN tumors from other gastrointestinal stromal tumors is based on electron microscopic findings. Thus far there have been 12 reported cases. We present an additional 12 GAN tumors, identified by us during 4 years. There were seven male and five female patients and they ranged in age from 10 to 85 years (mean: 58 years). Sites of the tumors were stomach (three), jejunum (two), ileum (four), mesentery (one), and retroperitoneum (two). Eight of the tumors measured > 10 cm in greatest dimension. Usually well circumscribed, the neoplasms were tan to light pink, sometimes hemorrhagic, and soft. There was a variety of histologic patterns including fascicles, palisades, and whorls. Mitotic activity varied from 0 to 23 mitosis per 10 high-power fields (HPF). Using a panel of 10 immunohistochemical stains, only vimentin was consistently positive. There was neuron-specific enolase reactivity in six and S-100 protein reactivity in two cases. All muscle markers were negative. Ultrastructural studies showed neuron-like cells with long axonic cytoplasmic processes ending in bulbous synapse-like structures containing dense-core neurosecretory granules and clear vesicles. Basement membrane was absent. These features are reminiscent of ganglia of the intestinal autonomic nervous system. The patients were followed for 5-125 months (mean of 26 months). Tumor recurred or metastasized to the liver in seven patients (58%) and four patients died with tumor. There were correlations between tumor size (> 10 cm), mitotic count (at least five per 10 HPF), and aggressive behavior.     

Muscle differentiation and clinicopathologic features of gastrointestinal stromal tumors

We analyzed 46 gastrointestinal stromal tumors (GISTs) using a panel of antibodies to determine the frequency of smooth muscle differentiation and the relationship of immunophenotype to histopathologic features and clinical behavior. Thirty-six GISTs were classified as benign or malignant based exclusively on clinical behavior; a 2-year minimum follow-up was required for benign lesions. GISTs were immunopositive in the following categories: vimentin 45 of 46, desmin nine of 45, muscle-specific actin (MSA) 36 of 46, alpha-smooth muscle actin (SMA) 34 of 46, chicken gizzard actin-7 zero of 38, cytokeratin two of 46, S100 protein six of 46, glial fibrillary acidic protein (GFAP) zero of 46, synaptophysin zero of 46, and chromogranin one of 46. At least one muscle marker was positive in 39 of 46 tumors. Five GISTs were MSA positive/SMA negative, and three were MSA negative/SMA positive. All desmin-positive cases reacted with MSA or SMA. Eight GISTs were positive for vimentin, MSA, SMA, and desmin, whereas seven were vimentin positive only. Compared with the latter, the former tended to be smaller, less often necrotic, and clinically benign (p less than 0.05 for each). All vimentin-positive only GISTs were malignant. Immunohistochemical features did not correlate with tumor site, cellularity, nuclear pleomorphism, or mitotic rate. Benign GISTs were less cellular than were malignant GISTs (p less than 0.05), but they did not differ statistically in degree of nuclear pleomorphism, necrosis, mitotic rate, or size. We conclude that (a) 85% of GISTs react with at least one muscle antibody; (b) immunohistochemical features are unrelated to anatomic site; (c) SMA is, in effect, as sensitive as MSA, whereas desmin is less sensitive; and (d) simultaneous vimentin, MSA, SMA, and desmin positivity correlates with a benign outcome.     

Mixed endometrial stromal and smooth muscle tumors of the uterus: a clinicopathologic study of 15 cases

Uterine tumors composed of a prominent component of smooth muscle (SM) and endometrial stroma (ES) (so-called stromomyomas) have received little attention in the literature. The features of 15 of these tumors, defined as those containing more than 30% of each component, were evaluated. Many of the tumors were referred because of problems in the differential diagnosis. Patient age ranged from 29 to 68 years (mean, 46 years). The tumors ranged from 3 to 27 cm (average 9.6 cm) in diameter, and most were grossly well circumscribed. The sectioned surfaces often had soft, tan-yellow areas admixed with firm, whorled areas. Microscopic evaluation disclosed that nine tumors were well circumscribed, and six had infiltrating tongues typical of endometrial stromal sarcoma (ESS). The endometrial stromal component, which predominated in five cases, typically was characterized by a diffuse growth of closely packed, minimally atypical small cells accompanied by numerous arterioles and was desmin-negative in all cases tested, except for rare desmin-positive cells in three tumors. Five tumors showed sex-cord-like differentiation in these areas. The smooth muscle component, which predominated in seven cases, was composed predominantly of spindle cells in disorganized short fascicles, longer fascicles, or nodules with prominent central hyalinization. This component appeared benign, except in one case with moderate cytologic atypia, focal tumor cell necrosis, and 4 mitotic figures/10 high-power fields. The smooth muscle component was strongly desmin-positive in all the tumors tested. Follow-up of more than 1 year was available for seven patients. Six patients were alive and well, but one tumor with infiltrative borders recurred at 48 months as a pure endometrial stromal sarcoma. Mixed endometrial stromal and smooth muscle tumors should be distinguished from highly cellular leiomyomas, pure endometrial stromal tumors, and "uterine tumors resembling ovarian sex cord tumors," at least until knowledge of their clinicopathologic features is more complete. For treatment purposes, these tumors should be reported as endometrial stromal nodules or as endometrial stromal sarcomas with smooth muscle differentiation and any unusual features of either component recorded in a notation.     

Sclerosing peritonitis associated with luteinized thecoma of the ovary

A unique case of bilateral luteinized thecomas of the ovary associated with sclerosing peritonitis is reported and the clinical and pathological features of this and previously reported cases are reviewed. The patient, 52 years of age, presented with abdominal distension and diarrhea. Pelvic imaging studies revealed bilateral ovarian tumors with ascites. Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adhesiotomy of the small bowel were performed. Histologically, the ovarian tumor was composed of closely packed spindle to round-shaped cells, and within the spindle cell population, lutein-like cells were scattered singly or in clusters. Mitotic counts of spindle cells revealed 12 mitotic figures (MF) per 10 high-power fields (HPF) in one part of the left ovarian tumor, but other areas of the tumor showed less than 3 MF/10 HPF on average. The lesion from the resected small bowel showed prominent fibrosis, confined to the serosa with no evidence of metastasis from the ovarian tumor. The patient has undergone adhesiotomy with partial resection of the small bowel seven times since the first laparotomy because of the recurrent small bowel obstruction. The patient has survived with complications due to short bowel syndrome for 7 years after the initial surgery and so far no recurrence or metastasis of the ovarian tumor has been identified. The case reported here also supports the idea that luteinized thecoma of the ovary associated with sclerosing peritonitis may be a distinct clinicopathologic entity, in terms of the unique association and of the unique features of thecoma; that is, bilateral, hormonally inactive and apparently benign in spite of its highly mitotic activity. Additional attention should be paid to the patient's quality of life, which is often degraded by peritoneal fibrosis and small bowel obstruction.     

Ovarian neoplasms in children

OBJECTIVE: To review the clinical presentation, treatment, and outcome in a series of children with ovarian neoplasms. DESIGN: A retrospective review of the medical records in a case series of 29 girls with ovarian neoplasms. The length of follow-up ranged from 6 months to 7 1/2 years and averaged 3.0 years in the girls with malignant tumors. SETTING: The patients were treated at a large referral children's hospital. PATIENTS: Twenty-nine girls with ovarian neoplasms were treated from 1976 to 1992. The average age of the patients was 10 years and ranged from 2 to 16 years. MAIN OUTCOME MEASURES: The principal outcomes examined were mortality and surgical morbidity. RESULTS: The most common presenting symptoms for these ovarian tumors in pediatric patients included chronic abdominal pain, an abdominal mass, or distention. Three girls presented with precocious puberty or hirsutism. In 27 cases, the tumor was a primary ovarian lesion. In two patients, the ovarian mass was the presenting finding for a stage IV non-Hodgkin's lymphoma. Seventeen tumors were benign and 12 were malignant. Tumors originating from the germ-cell line predominated (n = 17). Seven of the 10 ovarian malignant neoplasms were stage I at the time of diagnosis. All but one of the girls with malignant tumors received either adjunctive radiation therapy or multiple-agent chemotherapy. Two girls with sex cord/stromal cell tumors who presented with stage I disease ultimately developed widespread metastases. Both girls with large epithelial tumors survived. All of the girls with benign tumors and seven (70%) of 10 with malignant lesions survived. CONCLUSION: Ovarian tumors are unusual lesions in the pediatric population. Unlike in adults, such neoplasms generally originate from the germ-cell line. Whereas most ovarian tumors in girls are benign, some children have malignant tumors that are very aggressive and do not respond well to adjuvant therapy. In particular, malignant sex cord/stromal cell tumors, even when they present at an early stage, may behave unpredictably.     

Neurofibroma and cellular neurofibroma with atypia: a report of 14 tumors

The clinical significance of nuclear atypia in neurofibromas that lack necrosis or significant mitotic activity has not been systematically studied. We reviewed 14 neurofibromas from six patients with mild to marked nuclear atypia, with low mitotic activity in some tumors. Five tumors also had areas of increased cellularity consistent with cellular neurofibroma. Necrosis was absent. All patients were treated by conservative excision. Clinical follow-up, ranging from 8 months to 6 years, showed that none of the tumors recurred or metastasized. To further characterize these neoplasms, we assessed p53 expression, proliferation rate, and DNA content because these methods have been suggested by others as useful in differentiating benign from malignant nerve sheath tumors. p53 expression was detected by immunostaining in one tumor with 5% positive cells and in two tumors with rare positive cells (<1%). The remaining 11 tumors were negative. Tumor cell proliferation rate as determined by Ki-67 immunostaining showed <5% positive cells in 13 tumors. In one tumor, 10% of the cells were Ki-67 positive. Using flow cytometry methods and paraffin-embedded tissue, all tumors had diploid DNA content with an S phase fraction ranging from 5.2% to 18.2% (mean 9.4%). No significant differences were observed between the neurofibromas and cellular neurofibromas. For comparison, we studied three malignant peripheral nerve sheath tumors (MPNSTs). All MPNSTs had relatively high p53 (range 10-16%; mean 12%) and Ki-67 (range 32-42%; mean 38.0%) staining. One of the MPNSTs was aneuploid. The S phase fraction of the MPNSTs ranged from 8.1% to 51.8% (mean 28.6%). These results suggest that clinically benign neurofibromas, both usual and cellular types, can have significant cytologic atypia that can be accompanied by low mitotic activity. Conservative surgical excision for these tumors is adequate. The results of p53 and Ki-67 immunostaining and DNA content and S-phase analysis by flow cytometry support this interpretation. In addition, in tumors with borderline histologic findings, results of these ancillary studies may be useful in distinguishing benign from malignant nerve sheath tumors.     

Malignant granular cell tumor: report of a case and review of the literature

The histological, immunohistochemical and electron microscopic features of a rare malignant granular cell tumor (GCT) arising in the left radial nerve of a 54-year-old man are reported. Despite a lack of local recurrence following extirpation, the tumor metastasized to the skull five years later. Light-microscopically, both primary and metastatic tumors consisted of markedly atypical or pleomorphic neoplastic cells with abundant cytoplasm containing diastase-resistant periodic acid Schiff reaction-positive granules. These tumor cells were arranged in a sheet-like pattern with mitotic figures including atypical ones, and were frequently immunopositive for proliferating cell nuclear antigen and c-MET, the c-met proto-oncogene product. These findings reflect high-grade malignancy of the present tumor. In addition, the tumor cells were positive for S-100 protein and neuron-specific enolase. Ultrastructurally, a large number of intracytoplasmic granules featuring secondary lysosomes as well as long interdigitating cytoplasmic processes, intercellular intermediate junctions, discontinuous basal lamina-like structures, and stromal long-spacing collagen were observed. These findings indicated schwannian differentiation of the present tumor. In addition, based on a review of previously reported cases, the overall clinicopathological characteristics of malignant GCT were summarized.     

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma)

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle-aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer-like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and "pericytoma." The epithelioid leiomyoma, the benign form, often arises in the mid- and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, assoicated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.     

New histopathologic data of prognostic value in extra-adrenal paragangliomas. Study of 9 cases

BACKGROUND: The biological behavior of paragangliomas is difficult to evaluate by classic histological criteria thus justifying the use of immunohistochemical markers as prognostic factors. METHODS: Nine extra-adrenal paragangliomas (three jugulo-tympanic, four carotid-body tumors, and two retroperitoneal) were studied by conventional histological criteria, and also by chromogranin A and neuron-specific enolase (NSE) immunohistochemical staining for the study of chief cells, and S-100 as a marker of sustentacular cells. The rate of cell proliferation was studied by the proliferating cell nuclear antigen (PCNA). The correlation between these parameters and the clinical evolution of the neoplasms, which were classified as benign, locally aggressive, and malignant (with metastasis), were also analyzed. RESULTS: The atypia and the mitotic rate did not correlate with the behavior of the tumor. Less immunostaining with the anti-S-100 and anti-chromogranin A antibodies was observed in the malignant paragangliomas and in those which were locally aggressive. In the benign tumors the proliferative rate (PCNA) oscillated between 0.7% and 3.7%, and 40 or less PCNA positive cells were counted in 10 high-power field (HPF) (40x). In malignant and locally aggressive tumors the proliferative rate was 5% or more, with 60 or more cells that were positive for PCNA being found in 10 HPF. CONCLUSIONS: The histopathologic signs implying worse prognosis in extra-adrenal paragangliomas are a decrease in chromogranin A and S-100 immunoreactivity and a rate of cell proliferation of 5% or greater, or a number of cells stained for proliferating cell nuclear antigen greater than 50 in 10 high-power field.     

Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses

Small cell neuroendocrine carcinomas (SNECs) of the sinonasal tract are extremely uncommon tumors. We reviewed the clinicopathologic features of six cases of this neoplasm. There was no sex preponderance with three females and three males and a mean age at presentation of 51 years (range, 38 to 68). Two patients had disease limited to the nasal cavity, and in four the tumor involved the nasal cavity and maxillary or ethmoid sinuses. Involvement of the orbit was present in two patients. Surgery was the primary treatment. After a mean follow-up of 37 months, one patient died of local disease and liver metastases, four were alive with recurrent or metastatic disease, and one died of unrelated causes. The tumors were composed of sheets, nests, and trabeculae with extensive areas of necrosis and hemorrhage. The individual cells were small to intermediate in size and had scanty cytoplasm. The nuclei were oval or round and hyperchromatic with absent or inconspicuous nucleoli. Nuclear molding and crush artefact were present in five cases. All tumors had a high mitotic rate with frequent abnormal mitotic figures. All cases stained for Cam 5.2, neuron-specific enolase, and chromogranin. Five cases were positive for AE1:AE3, and four for synaptophysin. No case stained for S-100 protein, or neurofilaments. O-13 stained one case. No case contained EBV-RNA. SNECs of the nasal cavity and paranasal sinuses are aggressive tumors with pathological features similar to those of anaplastic small cell carcinomas of the lung. They exhibit morphological and immunophenotypic features different from olfactory neuroblastoma and should be distinguished from this tumor.     

Minimal-deviation endometrioid adenocarcinoma of the uterine cervix. A report of five cases of a distinctive neoplasm that may be misinterpreted as benign

We describe five cervical adenocarcinomas with unusual, deceptively benign histological features that occurred in women 34 to 42 years of age and caused problems in interpretation. The tumors were incidental findings in hysterectomy or cone-biopsy specimens in four patients; the fifth patient was investigated because abnormal glandular cells were found on a Papanicolaou smear. One patient had been exposed in utero to diethylstilbestrol. The cervix is known to have been abnormal on gross evaluation in only one case. Microscopic examination disclosed a deceptively benign-appearing proliferation of glands and cysts for the most part unassociated with a stromal reaction. Cilia were present in four neoplasms and apical snouts in three. Features that indicated the neoplastic nature of the glandular proliferation in these cases, to varying extents in individual cases, included the number of glands and their distribution, the shapes of the glands, their presence deep in the cervical wall, the focal presence of a stromal reaction, and moderate cytologic atypicality with occasional mitotic figures. None of the tumors is known to have recurred or metastasized. In our opinion, these distinctive neoplasms represent minimal-deviation endometrioid adenocarcinomas of the cervix.     

Mucoepidermoid carcinoma of the major salivary glands: clinical and histopathologic analysis of 234 cases with evaluation of grading criteria

BACKGROUND: The authors had previously conducted an investigation of minor salivary gland mucoepidermoid carcinoma, in which they demonstrated that certain clinical and histopathologic features were useful in predicting biologic outcome. The current study investigated the usefulness of these features in determining the prognoses of patients with mucoepidermoid carcinomas of the major salivary glands. METHODS: Clinical data and 15 histopathologic features were compared in 4 patient groups based on outcome after initial treatment. The outcome groups were 1) survival without disease, 2) survival with tumor recurrence only, 3) survival with metastasis, and 4) death related to tumor. A numeric score was assigned to each unfavorable histopathologic feature. Low grade tumors had scores of 0-4. Intermediate grade tumors scored 5 or 6. High grade tumors had scores higher than 6. RESULTS: Most patients (75%) were tumor free after the initial treatment. Twenty-one patients (9%) had local recurrence only, 12 (5%) demonstrated metastasis and survived, and 25 patients (11%) died of their disease. CONCLUSIONS: Clinical features associated with metastasis or death were more advanced age, tumor size, and preoperative symptoms. Histopathologic features that correlated with poor outcome were cystic component less than 20%, 4 or more mitotic figures per 10 high-power fields, neural involvement, necrosis, and anaplasia. All five of these histopathologic features demonstrated statistical prognostic significance when parotid gland tumors from Groups 1 and 4 were compared (P < 0.001). The point-based grading system demonstrated a statistically significant correlation with outcome for parotid tumors but not for submandibular tumors. The authors' findings indicate that patients with tumors of equal histopathologic grade have a better prognosis when their tumors are in the parotid gland than when their tumors are in the submandibular gland. Six of eight submandibular tumors that metastasized or resulted in death were low grade lesions, and none were high grade.     

Surgical treatment of primary neurogenic mediastinal tumors

In the years 1968-1989 out of 300 patients with surgically treated mediastinal tumors in 63 (21%) they were of neurogenic origin. Eighteen of these (29%) proved to be malignant. These tumors occurred in 23 males and 40 females, age range 14 months-67 years (mean 36 years). In all 45 patients with benign tumors and in 12 (66.7%) with malignant tumors the tumors were totally resected. In 4 patients the tumor was only partially resected in further two only a biopsy of the lesion was taken. During postoperative period one patient with a malignant tumor died. Ten (71.4%) patients survived 5 years with malignant lesions, while only 8 (61.5%) survived ten years. All patients that underwent resection of the benign tumor survived 10 years.     

Mantle cell lymphoma: a clinicopathologic study of 80 cases

Mantle cell lymphoma (MCL) is a relatively uncommon yet distinct type of malignant lymphoma whose clinical and pathological characterization has been limited by the small numbers of cases published to date. We studied 80 cases of MCL seen at a single institution over 7 years to determine both clinical and pathological prognostic factors. The patients in this study were predominantly male (70%) and older (mean age, 63 years) and presented with advanced-stage disease (88%). Extranodal involvement was common. Median overall survival (OS) was 43 months. Except for performance status, prognosis was not significantly influenced by clinical prognostic factors. Histologically, MCL architecture was classified as diffuse (78%), nodular (16%), or mantle zone (6%); the OS among these groups was identical. Increased mitotic activity (>20 mitotic figures per 10 high power fields), blastic transformation, and peripheral blood involvement at diagnosis also predicted for a worse outcome, but bone marrow involvement did not. The presence or absence of a translocation t(11; 14) by cytogenetic analysis or a bcl-1 rearrangement by Southern analysis did not significantly predict outcome. In summary, this study of 80 cases of MCL highlights its distinctive clinicopathologic features and shows that increased mitotic activity, blastic morphology, and peripheral blood involvement at diagnosis are prognostically important factors.     

Hürthle cell tumors

OBJECTIVES: a) To provide a clinicopathological profile of Hürthle cell neoplasms (HCT) in our experience. b) To evaluate if there are any differences in the clinical or morphological features between three HCT categories: benign, malignant and indeterminate. c) To examine the role of the clinical and morphological features in predicting the behavior of these neoplasms. METHODS: We reviewed the clinical reports of all patients with a histological diagnosis of HCT at our Hospital between 1981 and 1996. The final study group consisted of 25 cases. The neoplasms were divided into three categories on the basis of presence and degree of capsular and vascular invasion, marked nuclear atypia, tumour necrosis and pattern of growth. A series of clinical parameters were evaluated. RESULTS: Of the 25 tumors, 52% were morphologically classified as benign, 8% as indeterminate and 40% as malignant. Follow-up ranged from 10 months to 14.8 years or until death (average 3.8 years). There were four local recurrences (20%), three in the malignant group (30%) and one in the benign group (7.6%) (p = 0.15). One patient presented metastases and died because of tumor during the follow-up. Apart from capsular and vascular invasion and some aspects of therapy, no significant differences were found in the clinical and histological parameters analyzed between the three histological groups or between the groups with or without recurrence. CONCLUSION: We did not find any clinical or morphological parameter which can predict recurrence among these tumors. Our study further establishes the controversial issues surrounding the biological behavior of Hürthle cell neoplasms.     

Anaplastic pleomorphic xanthoastrocytoma

Well-documented cases of malignant degeneration in pleomorphic xanthoastrocytoma and of anaplastic pleomorphic xanthoastrocytoma are rare in the literature. We report 2 cases of pleomorphic xanthoastrocytoma, 1 of which demonstrated clear evidence of malignant degeneration in the absence of prior radiation therapy over an 18-year period. Both anaplastic tumors were characterized by foci of necrosis and increased mitotic activity (3 and 2 mitotic figures/10 high-power fields). Both tumors demonstrated focal positive staining for glial fibrillary acidic protein and showed marked reticulin deposition. An MIB-1 labeling index (marker of cell proliferation) in the initial low-grade-appearing tumor in case 1 was 0.1%. The recurrent tumor in case 1 had an MIB-1 labeling index of 4.9%, and the anaplastic tumor in case 2 had an MIB-1 labeling index of 5.4%. Significant cyclin D1 immunoreactivity was not observed in either anaplastic tumor. Two percent to 3% of tumor cells stained positive with p53 protein antibody in the recurrent anaplastic tumor in case 1. Although histology may not reliably predict aggressive behavior in pleomorphic xanthoastrocytomas, the presence of increased mitosis, necrosis, and increased cell proliferation labeling indices may be indicative of a higher grade tumor.     

Pain in the family

A case is reported of atypical glomus tumor occurring in the posterior inferior mediastinum of a 26-year-old woman complaining of severe back pain. The tumor was composed of atypical small, round tumor cells with scattered mitotic figures. In addition to sheet-like, diffuse proliferation of the tumor cells, some areas of the tumor contained small "glomoid" cells arranged in organoid and hemangiopericytomalike patterns. Immunohistochemically, many tumor cells were positive for muscle-type actins and a few cells were focally positive for desmin. Ultrastructural studies revealed smooth muscle features of tumor cells, that is, pinocytotic vesicles, external laminas, dense plaques, and occasional thin filaments with dense bodies. The patient remained well for 5 years and 4 months after the operation without additional radiation and chemotherapy. The tumor was diagnosed as an atypical, or low-grade malignant, glomus tumor morphologically. It seems important to recognize the presence of this type of tumor in sites other than extremities and to differentiate it from other malignant small, round cell tumors.