Five-year remissions of metastatic solid teratoma of the ovary

To date, only three 5-year survivors with metastatic solid malignant teratoma of the ovary have been reported. The authors report two additional cases surviving 5 years or greater. In both cases, the embryonal teratomatous elements matured to Grade 0 peritoneal metastases after intensive chemotherapy and surgery with or without radiotherapy. Since only one of the two patients had an objective response to therapy, we speculated that the maturation of the tumor in both patients might be, but is not proven to be, a consequence of chemotherapy, surgery, and/or radiotherapy. Because the prognosis, even in Stage IA solid ovarian teratoma, is so poor, we suggest that all such patients should have 2 years of adjuvant chemotherapy consisting of vincristine, dactinomycin, and cyclophosphamide. Patients with demonstrable metastases should receive the same chemotherapy until either all tumor has regressed or all immature elements have reverted to mature tissue.     

Pure immature teratoma of the ovary: analysis of 22 cases

Between 1970 and 1991, 22 patients with pure immature teratoma were treated at the Center of Oncology in Krakow. Sixteen (72.7%) patients had stage I, four (18.2%) stage II, and two (9.1%) stage III of disease, nine (40.9%) patients had grade 1, 11 (50%) grade 2, and two (9.1%) grade 3 tumors. Eight stage Ia, grade 1 patients were treated with surgery only, the remaining 14 (63.6%) received postoperative chemotherapy. Five-year NED (no evidence of disease) survival was achieved in 81.8% of patients. Out of 16 stage I patients, 15 (93.8%) survived 5-year NED, out of six stage II and III, three (50%) patients only survived this period. We cured all grade 1 patients, and 81.8% (9/11) grade 2; two grade 3 patients died because of tumors. We also cured all six stage Ia patients, treated with unilateral salpingo-oophorectomy (with or without chemotherapy), and all eight stage Ia grade 1 patients treated with surgery only.     

A compound nevus in a benign cystic teratoma of the ovary

The occurrence of a compound nevus in an ovarian benign cystic teratoma is presented. This appears to be the first report of this lesion in the ovary without coincident melanoma. The case illustrates a probable origin for ovarian melanoma and raises a question concerning the origin of the melanocyte.     

Thyroid carcinoma originating in a teratoma of the ovary

The malignant degeneration of a cystic teratoma of the ovary is a rare complication which different authors place at between 0.8 to 4%. Adenocarcinomas represent a relatively rare complication, 8% of secondary cancers, those of thyroid origin being reported in only 5 cases. The aim of this paper is to present the sixth such case of thyroid carcinoma developed in a mature teratoma in the ovary.     

Morphologic prognostic factors of malignant mixed müllerian tumors of the ovary: a clinicopathologic study of 15 cases

Interleukin-2 (IL-2)-based therapy induces a vascular leak syndrome (VLS), manifested by hypotension, tachycardia, and oliguria, as is also seen with septic shock. The optimal method for treating such VLS is not known. A prospective randomized trial was undertaken to compare crystalloid and colloid fluid resuscitation for patients receiving bolus IL-2-based therapy for metastatic cancer. All patients received maintenance crystalloid fluid administration and were randomized to receive crystalloid (0.9% normal saline) or colloid (5% human serum albumin) fluid boluses to maintain acceptable vital signs and urine output. Patients refractory to fluid boluses were given dopamine for oliguria and/or phenylephrine for hypotension. Of 107 patients who completed one cycle of therapy on study, 76 completed a full treatment course (two cycles) on study. The total number of saline and albumin fluid boluses given were 9.5 +/- 0.9 versus 7.7 +/- 0.7 (p = 0.36, n = 107) for the first cycle and 19.2 +/- 1.8 versus 16.1 +/- 1.6 (p = 0.33, n = 76) for a complete course, respectively. Although patients receiving saline boluses had significantly more oliguria during a course of therapy, weight gain, number of IL-2 doses, tachycardia, hypotension, vasopressor use, hospital stay, and clinical response rates did not significantly differ between arms. Changes in hematocrit, hemoglobin, protein, albumin, blood urea nitrogen (BUN), and creatinine were analyzed, and patients receiving crystalloid showed greater decreases in albumin (p < 0.0001) and total protein (p < 0.05) as expected. A 40-fold greater cost associated with albumin suggested that crystalloid resuscitation be used to treat the VLS associated with IL-2 therapy.(ABSTRACT TRUNCATED AT 250 WORDS)     

Lymphomatoid granulomatosis: A clinicopathologic study of four cases

Four cases of lymphomatoid granulomatosis were studied. One case, previously reported, has had a prolonged remission of 8 years' duration. In one case, the course was rapid and progressive, and the patient died 2 months after the onset of the disease. In two other patients, the disease appears to be arrested effectively, both clinically and radiographically, by administration of corticosteroids. In the fatal case, postmortem examination revealed a typical angiocentric and destructive polymorphous lymphoreticular infiltrate in the lungs, kidneys, and adrenal glands. The diagnosis was made on specimens obtained from three patients by open thoracotomy. An adequate specimen is mandatory for diagnosis and thoractomy is indicated. To be considered in the differential diagnosis are Wegener's granulomatosis, the limited form of Wegener's granulomatosis, lymphoma, allergic granulomatosis, lymphocytic interstitial pneumonia, plasma cell granuloma, and infectious granuloma.     

Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases

OBJECTIVE: To describe a clock drawing task (CLOX) designed to elicit executive impairment and discriminate it from non-executive constructional failure. SUBJECTS: 90 elderly subjects were studied (45 elderly and well persons from the independent living apartments of a continuing care retirement community and 45 patients with probable Alzheimer's disease). The clock drawing performance of elderly patients was compared with that of 62 young adult controls. METHODS: Subjects received the CLOX, an executive test (EXIT25), and the mini mental state examination (MMSE). The CLOX is divided into an unprompted task that is sensitive to executive control (CLOX1) and a copied version that is not (CLOX2). Between rater reliability (27 subjects) was high for both subtests. RESULTS: In elderly subjects, CLOX subscores correlated strongly with cognitive severity (CLOX1: r=-0.83 v the EXIT25; CLOX2: r=0.85 v the MMSE). EXIT25 and MMSE scores predicted CLOX1 scores independently of age or education (F(4,82)=50.7, p<0.001; R2=0.71). The EXIT25 accounted for 68% of CLOX1 variance. Only the MMSE significantly contributed to CLOX2 scores (F(4,72)= 57.2, p<0.001; R2=0.74). CLOX subscales discriminated between patients with Alzheimer's disease and elderly controls (83.1% of cases correctly classified; Wilkes' lambda=0.48, p<0.001), and between Alzheimer's disease subgroups with and without constructional impairment (91.9% of cases correctly classified; Wilkes' lambda=0.31, p<0.001). CONCLUSIONS: The CLOX is an internally consistent measure that is easy to administer and displays good inter-rater reliability. It is strongly associated with cognitive test scores. The pattern of CLOX failures may discriminate clinical dementia subgroups.     

Malignant melanoma of the choroid with balloon cells a clinicopathologic study of three cases

We report the findings on clinicopathologic examination of three patients with balloon cell melanoma of the choroid. Ophthalmoscopically, all three had small, slowly progressive tumors at the posterior pole, with an encircling yellow halo. With fluorescein angiography, the yellow halo showed fluorescence similar to the remainder of the tumor, thus differentiating this substance from lipofuscin pigment, drusen and exudates. Light microscopy demonstrated prominent balloon cells which were most numerous at the tumor margins. These cells showed variable melanin pigmentation and were negative for lipid, acid mucopolysaccharide, and glycogen. Special enzyme studies (lactic dehydrogenase, succinic dehydrogenase, acid phosphatase, beta glucoronidase and aminopeptidase) demonstrated some similarity to melanocytic cells. Electron microscopy revealed premelanosomes and complex melanosomes in the cytoplasm of balloon cells without evidence of significant lipid.     

Partial moles: a clinicopathologic study of 25 cases

This study is based on a review of all moles diagnosed at the Kaplan Hospital in Israel from 1968 to 1977. Histologic reevaluation revealed that of a total of 72 moles, 47 (65%) were complete and 25 (35%) partial. In contrast to complete moles, about one third of the partial moles showed fetal parts. An analysis of the patient records showed smaller uteri, less vaginal bleeding, absence of severe vomiting, lower gonadotropin levels, and normal follow-up in patients with partial moles as compared with those who had complete moles. These results indicate that the partial mole is a distinct clinicopathologic entity that can be suspected by the clinician and confirmed by the pathologist on morphologic grounds, even in the absence of cytogenetic analysis.     

Salivary duct carcinoma: a clinicopathologic study of three cases with a review of the literature

Three cases of salivary duct carcinoma are presented. They occurred in a 60 year old man, a 66 year old man and a 57 year old woman. All of the lesions were located in the parotid gland. The tumor size ranged from 3 to 5 cm across the largest diameter. Facial paralysis was observed in two cases. Histologically, intraductal and invasive adenocarcinoma showing papillary, cribriform, and solid patterns with comedolike necrosis was observed. Immunohistochemically, the tumor cells were positive for keratin and epithelial membrane antigen. No myoepithelial cells were demonstrated within the tumor by staining for S-100 protein, alpha-smooth muscle actin or muscle specific actin. Ultrastructurally, intracytoplasmic lumina with microvilli, a moderate number of mitochrondria, lysosomes, and tight junctions were found. Regional lymph node metastasis was observed in one case, and distant metastasis developed in two cases. All of the patients were treated with adjuvant postoperative irradiation. One patient died of disease at 11 months after the initial diagnosis, another was alive with disease at 8 months, and the third patient was alive without disease at 2 years and 3 months. Salivary duct carcinoma should be differentiated from low-grade salivary gland carcinomas using morphologic and clinical criteria because of its poor prognosis even with aggressive therapy.     

Hemangiopericytoma-like intranasal tumors. A clinicopathologic study of 23 cases

The clinical, microscopic, and gross pathologic features of 23 cases of intranasal hemangiopericytoma-like tumors are reviewed and studied. When in the nasal cavity, these lesions often originated in a paranasal sinus and extended into the nasal cavity secondarily. They occurred most commonly in adults in the sixth and seventh decades of life; there was no significant sex predilection. Twenty-two of the 23 patients were Caucasian. These patients most commonly had symptoms of nasal obstruction and epistaxis. Clinically the lesions were generally thought to represent allergic polyps. Although appearing microscopically as non-differentiated spindle-cell neoplasms, these lesions showed little nuclear or cytoplasmic pleomorphism, minimal mitotic activity, and no necrosis or hemorrhage or other evidence of anaplasia found in malignant tumors. Follow-up data showed no evidence to suggest a malignant or biologically unpredictable lesion. Nineteen of 22 cases followed showed no recurrence regardless of the treatment; those that recurred did so locally. No metastasis or other form of aggressive behavior attributed to hemangiopericytomas in other anatomic locations was seen in this series. Another case, diagnosed as a malignant hemangiopericytoma of the nasal cavity, showed dissimilar and anaplastic histologic features. This case metastasized and is discussed, though not included in this study.     

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.     

A clinicopathologic study of the resected cases of adenosquamous carcinoma of the lung

Low-grade malignant tumors (LGMT) of the lung were surgically treated in our institute between 1981 and 1997. Both the characteristics and prognosis were examined. We studied 10 cases with LGMT of the lung, male to female ratio 1:1, age range 15 to 71 years, mean 55 years, 6 central and 4 peripheral. Five patients had lobectomy, 3 had sleeve lobectomy and 2 had bilobectomy. Pathologically, 5 samples were typical carcinoid, 3 were mucoepidermoid carcinoma and 2 were adenoid cystic carcinoma. None of the patients had lymph node metastasis. Nine patients were stage I and one stage IIIA. Seven patients underwent absolutely curative resection; 2, relatively non-curative resection; 1, non-curative resection. In preoperative examination, only 4 patients was diagnosed correctly and the others were misdiagnosed. Mean survival time was 167.5 months. One patient died from causes other than the primary cancer and the others are surviving (range 2-173 months). The LGMT group was significantly younger and had a significantly better prognosis compared with the control group (p=0.02). Mediastinal lymph node dissection is suggested to be omitted. However, further accumulation of cases is necessary regarding to the omission of lymph node dissection.     

Intravenous leiomyomatosis of the uterus: a clinicopathologic study of 22 cases

Sphingosine-1-phosphate (Sph-1-P) has been implicated as a second messenger in control of cell motility and proliferation (e.g., Sadahira Y, et al., PNAS 89:9686, 1992; Olivera A & Spiegel S, Nature 365:557, 1993). The control mechanism for its synthesis, as catalyzed by sphingosine kinase, is crucial in signal transduction. Synthesis of Sph-1-P in Balb/c 3T3 fibroblasts (A31 variant) is strongly up-regulated by brief treatment of cells with 12-O-tetradecanoylphorbol-13-acetate (TPA). Level of Sph-1-P in PKC-depleted cells is 10-fold higher than in undepleted cells, and a further 5-fold increase occurs after treatment with TPA. In Swiss 3T3 and B16 melanoma cells, Sph-1-P level was unaffected by TPA treatment. Thus, the effect of TPA on Sph-1-P synthesis appears to be cell type-specific.     

Mucoepidermoid carcinoma of the conjunctiva: a clinicopathologic study of five cases

Five patients with mucoepidermoid carcinoma of the conjunctiva were studied. The tumors were located close to the limbus in three patients, in the bulbar conjunctiva in one, and in the lower cul-de-sac in the remaining patient. Histopathologically, they exhibited lobules of tumor cells showing an admixture of epidermoid and mucus-secreting cells. Histochemical stains for mucin were most useful in arriving at a correct diagnosis. Follow-up information revealed that all five tumors recurred rapidly, invading the corneal stroma, intraocular structures, and orbit. Biologically, these tumors appear to be locally aggressive and should be differentiated histopathologically from the conventional squamous cell carcinoma of the conjunctiva, which carries a better prognosis. They should be managed by wide local excision, and the margins should be carefully examined for residual tumor. Frequent follow-up is recommended to detect early recurrence.     

Thyroid paraganglioma: a clinicopathologic and immunohistochemical study of three cases

We report three cases of intrathyroidal paraganglioma. The patients were adult women without significant personal or family histories that presented with an asymptomatic thyroid nodule. The tumors were single, well-circumscribed solid masses, 2 cm in greatest diameter, located within one thyroid lobe. Microscopically, they were encapsulated and showed the typical nesting (Zellballen) pattern of paraganglioma in other sites. Two of the tumors were composed of small- to medium-sized cells with granular amphophilic cytoplasm, and the third consisted of relatively large cells having a similar staining quality. Immunohistochemically, all tumors showed positivity for neuron-specific enolase, chromogranin A, and synaptophysin. S-100 protein-positive sustentacular cells were demonstrated in each case. Negative staining for epithelial markers, thyroglobulin, carcinoembryonic antigen, calcitonin, calcitonin gene-related peptide, serotonin, vimentin, and Congo red excluded other tumors that were considered in the differential diagnosis, such as medullary carcinoma, hyalinizing trabecular adenoma, atypical follicular adenoma, Hurthle-cell neoplasm, and metastatic carcinoid tumor. The patients were alive and well without evidence of recurrent disease at the time of the last follow-up. The previous literature on these tumors is discussed. We conclude that intrathyroidal paraganglioma exists and that this tumor can be distinguished from other similar-appearing neoplasms in this organ.     

A clinicopathologic study of thymomas: a review of 52 cases, with particular reference to results of treatment

The possible use of spleen-derived mononuclear cells (SPMC) for the intentional and economical adoptive immunotherapy of cancer patients was studied. SPMC were obtained from spleens resected surgically from patients with gastric cancer or idiopathic thrombocytopenic purpura (ITP). When SPMC were cultured in recombinant interleukin 2 (rIL2), SPMC, in the form of interleukin-activated killer spleen cells (IL-SP) proliferated in six of eight cases. CD8+ lymphocytes were the major expanding cell population in most SPMC cultures and IL-SP showed a significant cytolytic activity against cultured tumor cells during cell proliferation. When cultured with a streptococcal preparation, OK-432, for 24 to 48 h, SPMC showed cytotoxic activity against tumor cells and were expressed as OK-432 activated killer spleen cells (OK-SP). The effects of supernatants from IL-SP and OK-SP on tumor cell growth were also examined. The supernatants from IL-SP and OK-SP significantly inhibited cell growth in 3 and 10 out of 11 cases, respectively, while those from OK-SP showed higher growth inhibitory activity than those from IL-SP. The results of this study indicate the potential of SPMC as effector cells for the adoptive immunotherapy of cancer patients.     

Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases

BACKGROUND: In order to improve management, the files and tissue sections of 28 cases of malignant peripheral nerve sheath tumors (MPNST) diagnosed at the University of Virginia Health Sciences Center between 1960 and 1990 were reviewed. METHODS: Clinical data tabulated included age, sex, race, the presence or absence of von Recklinghausen neurofibromatosis type 1 (NF-1), tumor size, tumor location, type of treatment, and status of surgical margins. Pathologic study included assessment of mitotic rate, divergent differentiation, cellular atypia, necrosis, and vascular reaction. RESULTS: The median disease-free survival time was 11 months, and the median overall survival time was 44 months. Overall survival and disease-free survival were significantly influenced by patient age, tumor location, tumor size, extent of surgery, and quality of margins. Patients with a family history of neurofibromatosis also had better disease-free survival. None of the other clinical variables correlated with survival. CONCLUSIONS: The authors recommended that patients with NF-1 be followed closely for MPNST development. For most cases, treatment should include aggressive surgery with wide surgical margins combined with adjuvant radiation therapy. Chemotherapy may have a role for treatment failures.     

Stromal tumors of the jejunum and ileum: a clinicopathologic study of 39 cases

Recently, cell size, cell density, and growth pattern were found to be reliable histologic parameters in separating benign from malignant duodenal stromal tumors. However, there are few data on the histologic features and important prognostic parameters of stromal tumors from other parts of the small bowel. Thus, we studied the clinical and pathologic features of 39 stromal tumors of the jejunum and ileum to determine which parameters would be most useful in distinguishing a benign from a malignant tumor. In all cases, the following histologic parameters were recorded: (a) predominant growth pattern (organoid, fascicular, solid, or mixed), (b) cellularity (low or high), (c) predominant cell type (spindled, epithelioid, or mixed), (d) nuclear pleomorphism (minimal, moderate, or severe), (e) the presence or absence of tumor cell necrosis, (f) the presence or absence of mucosal infiltration, (g) the presence or absence of skeinoid fibers, and (h) the number of mitotic figures per 50 high-power microscopic fields (HPF). Clinical follow-up was obtained in all cases, and the patients were considered to have suffered an adverse outcome if they developed metastatic disease or died as a complication of their tumor. In the absence of these features, patients were not considered to have suffered an adverse outcome. Twenty-five patients suffered an adverse outcome. Twenty-one patients died of disease from 1 month to 9 years (median: 2 years). One patient died at 4 days because of postoperative complications. Three patients were alive with metastatic disease at 6 months, 6 years, and 7 years. Twenty-four of these 25 patients developed metastatic disease, most commonly to the liver. Fourteen patients did not suffer an adverse outcome. Eleven patients were alive without disease from 2 to 11 years (median: 3 years), and three patients died of unrelated causes at 1, 1, and 3 years. Although there was some overlap in features between clinically benign and malignant tumors, features that were significantly associated with an adverse outcome included tumor size > 5 cm, mitotic counts > 5 mitotic figures per 50 HPF, high cellularity, the absence of a predominant organoid growth pattern, the absence of skeinoid fibers, the presence of severe nuclear pleomorphism, and the presence of mucosal infiltration and tumor cell necrosis (p < 0.05 using the chi-square and Fisher's exact tests). Features that were significantly associated with decreased survival included tumor size > 5 cm, mitotic counts > 5 mitotic figures per 50 HPF, high cellularity, the absence of skeinoid fibers, and the presence of tumor cell necrosis (p < 0.05 using the Mantel-Haenszel log-rank test). Given the fact that there is some overlap in these features between clinically benign and malignant tumors, a multiparametric analysis using the above features is the most effective way of predicting clinical behavior.