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Biomedical subjects

E E Lack

Publications and source records attributed to E E Lack.

At least 19 recordsLinked to original sources

Cardiac papillary fibroelastoma: an immunohistochemical investigation and unusual clinical manifestations.

Cardiac papillary fibroelastoma (CPF) is a morphologically distinctive, but rare, cardiac lesion that is usually found incidentally at autopsy or during open heart surgery. Because of improved diagnostic imaging techniques, the premortem or preoperative diagnosis of CPF is becoming more frequent. The histogenesis of CPF, however, remains controversial. Herein we report an immunohistochemical investigation of 11 cases of CPF; two cases showed unusual embolization phenomena, including one with histologically documented pulmonary arterial embolism. For comparison, nine cardiac myxomas (CMs) and eight examples of organizing thrombi were also studied. Immunohistochemical markers included keratin, vimentin, collagen type IV, muscle-specific actin, desmin, factor VIII-related antigen, CD34, and S-100 protein. The cells covering the surface of both CPFs and CMs were positive for vimentin, factor VIII-related antigen, and CD34, in keeping with their presumed vascular endothelial origin. Interestingly, the surface lining cells were also positive for S-100 protein in all CPF and in eight of nine CMs. In CPF, collagen type IV showed multilayered linear staining beneath the surface that was virtually identical to the staining pattern for elastic tissue. The major immunophenotypic difference between CPF and CM is the frequent presence of muscle-specific actin in the stellate cells of the stroma in CM but not in CPF. Although this study did not clarify whether CPF is a hamartomatous, neoplastic, or reparative process, it demonstrated active participation of the surface endothelial lining cells with excessive formation of basal membrane material in the formation of CPF.

Adult

Diffuse hemorrhagic gastroenteropathy: report of a new entity.

An apparently novel entity, diffuse hemorrhagic gastroenteropathy (DHG), in a 70-year-old female who had an unremitting course of chronic gastrointestinal blood loss for 3 years requiring transfusion of more than 200 units of packed red blood cells over this period is reported here. Endoscopy showed diffusely hemorrhagic mucosa in the stomach, duodenum, and small bowel. Full-thickness biopsy of the stomach and small intestine revealed luminal narrowing of capillaries and post-capillary venules within the lamina propria due to swelling and some proliferation of the endothelial cells with margination and emigration by neutrophils as well as partial occlusion of some vessels by fibrin thrombi. DHG may represent a new entity characterized by mucosal hemorrhage due to local mucosal ischemia of the gastrointestinal tract secondary to a small vessel "vasculopathy" apparently restricted to this site.

Aged

Primary lymphoma of the appendix. Case report and review of the literature.

Malignant lymphomas comprise 1-4% of the malignant neoplasms of the gastrointestinal tract, but appendiceal lymphomas are exceedingly rare. Herein is presented a case of a well differentiated lymphocytic lymphoma of the appendix found incidentally at hernia repair. Forty-six cases of appendiceal lymphoma have been reported since 1898 with a mean patient age of 25.7 years. Thirty-one patients presented with right lower quadrant pain, and a mass was an incidental finding in five. Of the 46 cases, follow-up was possible in 28. There were four deaths within 30 days of the operation and five deaths within 1 year. Although extensive follow-up is limited, there have been only two reported deaths secondary to primary appendiceal lymphoma since 1945 and these two cases are discussed in detail. Based on this extensive review, appropriate recommendations are made.

Aged

Cytokeratin expression in adrenocortical neoplasia: an immunohistochemical and biochemical study with implications for the differential diagnosis of adrenocortical, hepatocellular, and renal cell carcinoma.

The immunostaining patterns of adrenocortical tumors are not clearly defined, primarily due to their inconsistent expression of cytokeratins (CK). To address this issue and to investigate whether adrenocortical tumors can be immunohistochemically differentiated from histologically similar tumors arising from the kidney and liver, we studied four normal adrenal glands, two adrenocortical adenomas (ACAs), 31 adrenocortical carcinomas (ACCs), 37 renal cell carcinomas (RCCs), and 33 hepatocellular carcinomas (HCCs) with anti-CK antibodies AE1, CAM 5.2, UCD/PR10.11, 35BH11, PKK1, and Ks19.1, as well as antibodies to vimentin (VIM), epithelial membrane antigen (EMA), and HMFG-2. Normal adrenal cortical cells showed variable staining with all anti-CK antibodies on fixed and frozen sections. In contrast, only one of two fixed ACAs stained with a single anti-CK, although both neoplasms reacted with multiple anti-CK antibodies on frozen sections. Similarly, 20 of 31 fixed ACCs contained VIM, but only one tumor stained for CK; frozen sections of this and another, previously negative tumor, however, stained with most of the anti-CK antibodies tested. One-dimensional Western immunoblot analysis confirmed the presence of CKs 18 and 19 in two examples of normal adrenal cortex, one ACA, and the ACC immunohistochemically positive on fixed and frozen sections, with CK 19 identified in the ACC that was positive on frozen section alone. All fixed HCCs and most RCCs stained with multiple anti-CK antibodies (33 and 34 cases, respectively), with a proportion of tumors positive for VIM (six and 22 cases, respectively), EMA (seven and 30 cases, respectively), and HMFG-2 (15 and 28 cases, respectively). The results suggest that CK expression is diminished in most adrenocortical tumors to levels too low to be recognized following the deleterious effects of fixation. While the immunohistochemical absence of CK, EMA, and HMFG-2 in fixed sections in the majority of ACCs is distinctive, sufficient phenotypic overlap exists such that differentiation between RCC and HCC may not be possible in an individual case.

Adrenal Cortex Neoplasms

Amebiasis complicating carcinomas: a diagnostic dilemma.

Two black African women and one black American man had carcinomas of cervix, perineum, and sigmoid colon, respectively. In each of these patients, trophozoites of Entamoeba histolytica had invaded the surface of the tumor, and in some areas had invaded more deeply into the stroma between the tumor cells. Although it is well known that cutaneous amebiasis of anus, penis, vulva, and cervix can mimic squamous cell carcinoma, it may be, perhaps, less well known that carcinomas at these sites may be colonized by trophozoites of E. histolytica. In patients with amebiasis but without an associated carcinoma, a correct diagnosis of amebiasis spares the patient unnecessary and sometimes mutilating surgery. But a diagnosis of amebiasis, when there is an unrecognized underlying carcinoma, delays effective treatment of the carcinoma. A smear that establishes a diagnosis of cutaneous amebiasis, therefore, should be followed by biopsy to exclude or confirm an underlying carcinoma.

Adenocarcinoma

"Signet Ring" sinus histiocytosis mimicking metastatic adenocarcinoma: report of two cases with immunohistochemical and ultrastructural study.

The axillary lymph nodes from two patients with invasive breast carcinoma showed "signet ring" histiocytosis with sinusoidal distribution of cells having prominent cytoplasmic vacuolization which simulated metastatic adenocarcinoma. Immunohistochemical studies confirmed the histiocytic nature of the signet ring cells and eliminated the possibility of metastatic adenocarcinoma. Electron microscopy in one case revealed lipid within the cytoplasmic vacuoles. The etiology of this reactive change is unknown; however, a history of previous surgery involving the chest wall may be causally related with disruption of adipose tissue. Prior to ipsilateral mastectomy both patients had undergone a contralateral mastectomy for mammary carcinoma. Signet ring sinus histiocytosis is a reactive sinusoidal proliferation that can mimic metastatic carcinoma, but close inspection will usually reveal the histiocytic nature of the cells.

Adult

Primary osteosarcoma of the uterus: report of a case with immunohistochemical study.

A 63-yr-old female presented with uncontrollable uterine bleeding. Pathological evaluation revealed a uterine mesenchymal neoplasm which histologically represented a primary osteosarcoma. Immunohistochemistry was utilized to help support the diagnosis. The English literature regarding this rare uterine neoplasm is briefly reviewed.

Biomarkers, Tumor

Heterotopia of gastric mucosa and liver involving the gallbladder. Report of two cases with literature review.

Two cases of heterotopic tissue involving the gallbladder are presented (one gastric mucosa, the other liver), and the relevant literature is reviewed highlighting problems in differential diagnosis and complications, particularly with regard to gastric mucosal heterotopia. A firm diagnosis of gastric heterotopia is based on the presence of fundic mucosa replete with parietal and chief cells; a clear distinction from intestinal metaplasia should be made, but at times may be difficult. Potential complications include mucosal ulceration, obstruction, and hemorrhage. Of 39 patients presented, about half were 30 years of age or younger, and only nine have had calculi noted in the cholecystectomy specimen. Heightened awareness of gastric heterotopia with separation of smooth-muscle bundles may help to avoid a malignant diagnosis. Hepatic tissue involving the gallbladder that is completely separate from the main part of the liver is an even rarer form of heterotopia that should be distinguished from an accessory lobe.

Adult

Prolactin-producing pituitary carcinoma with pulmonary metastases.

Pituitary adenomas rarely are metastatic. Extracranial visceral metastases of prolactinomas were not previously reported. The authors report a case of a 34-year-old man with a prolactin-producing pituitary carcinoma and histologically proven lung metastases. Pathologic examination of the pulmonary spread included electron microscopy and immunohistochemistry; these confirmed prolactin production by the tumor. The patient's presentation at initial diagnosis, disease recurrence, clinical course, management, and response to therapy (with its theoretic basis) are detailed. Despite the use of dopamine analogues (to tolerance and in combination), there was documented intracranial and extracranial disease progression. Possible future therapeutic maneuvers are discussed.

Adult

Improved survival for children with anaplastic Wilms' tumors.

Combined modality treatment has resulted in cure rates of approximately 80% for children with Wilms' tumor. According to the National Wilms' Tumor Studies (NWTS), a group of patients with histologic features of anaplasia or sarcomatous Wilms' tumors (malignant rhabdoid tumors and clear cell sarcomas) were less responsive to vincristine and actinomycin. The survival rate of patients in this group with unfavorable histologic conditions was 54% compared with 90% for those with favorable histologic conditions. We have reviewed 80 consecutive cases of Wilms' tumor treated with a minimum follow-up interval of 5 years. Two pathologists independently reviewed all histologic specimens that were initially classified as having unfavorable histologic conditions and specimens from children with favorable histologic conditions who subsequently relapsed. One of 13 children with favorable histologic conditions had recurrent disease that was found to have unfavorable histologic conditions on rereview. All five patients with sarcomatous Wilms' tumor had a rapidly progressive course. Treatment of eight children with anaplastic Wilms' tumor with vincristine, actinomycin, cyclophosphamide, and abdominal radiation resulted in good disease-free and overall survival rates (5-year survival rate, 87.5%) that were not significantly different from children with tumors having favorable histologic conditions (5-year survival rate, 94%). All children with sarcomatous histologic conditions, however, did not to respond.

Antineoplastic Combined Chemotherapy Protocols

Intra-abdominal "angiosarcomatosis" report of two cases after pelvic irradiation.

Angiosarcomas account for only 1% to 2% of all soft tissue sarcomas with the most common site of origin being skin and subcutaneous tissue particularly on the face and scalp of elderly patients. These neoplasms rarely arise in the small or large bowel with presentation as diffuse abdominal "angiosarcomatosis." Two patients are reported who were treated with adjuvant pelvic irradiation for gynecologic malignancies (squamous cell carcinoma of the cervix and adenocarcinoma of the endometrium) in whom diffuse abdominal angiosarcoma of intestinal origin developed 7 and 18 years later. Both patients were treated with standard megavoltage irradiation. The clinical course was rapid in both cases with death due to intra-abdominal hemorrhage and bowel obstruction. The pertinent literature relating to the association between radiation therapy and subsequent development of angiosarcomas of soft tissues and other sites is briefly reviewed.

Adenocarcinoma

Müllerian adenosarcoma of the uterus with pure angiosarcoma: case report.

We report a case of müllerian adenosarcoma of the uterus with benign endometrioid epithelial component and histologically pure angiosarcoma. This seemingly unique sarcomatous ingredient, among uterine and extrauterine adenosarcomas reported in the English literature, is considered to be a homologous component of the tumor. The patient, a 71-year-old woman with a several-month history of intermittent vaginal bleeding, was found at abdominal hysterectomy to have a large polypoid intrauterine tumor with superficial extension into the myometrium. The patient was treated with 6,000 rad of adjuvant radiation but died a few months after of what clinically appeared to be massive abdominal bleeding. The literature regarding uterine tumors classified as angiosarcoma or malignant hemangioendothelioma is briefly reviewed.

Aged

Anaplastic thyroid carcinoma with osteoclast-like giant cells. A clinicopathologic, immunohistochemical, and ultrastructural study.

Two anaplastic thyroid carcinomas with osteoclast-like giant cells (OCL-GC) are reported. Light microscopically, one case resembled an aneurysmal bone cyst with cellular connective-tissue septae separating cavernous, blood-filled sinuses. The second case had sheets of anaplastic cells and a separate focus of papillary carcinoma without areas of transition. Multinucleate OCL-GC, pleomorphic mononuclear cells, and histiocytoid mononuclear cells with nuclei similar to those within the OCL-GC were seen in each case. With formalin-fixed, paraffin-embedded tissue, the majority of OCL-GC and histiocytoid mononuclear cells in both cases showed immunoreactivity using monoclonal antibodies to vimentin and KP-1, with the latter preparation directed against cells of monocytic/histiocytic lineage. Staining for keratin, epithelial membrane antigen, neuron-specific enolase, chromogranin, calcitonin, and thyroglobulin was negative in all cell types. Ultrastructural examination of one case showed two distinct types of mononuclear cells--one with morphologic characteristics similar to those of the OCL-GC, and another with pleomorphic nuclei and short cytoplasmic extensions joined by poorly formed desmosomes. The findings indicate that the anaplastic thyroid carcinomas studied represent poorly differentiated epithelial tumors infiltrated by reactive OCL-GC of monocytic/histiocytic lineage apparently derived from histiocytoid mononuclear cells via cellular fusion.

Carcinoma

Primary leiomyosarcoma of adrenal gland. Case report with immunohistochemical and ultrastructural study.

A primary leiomyosarcoma of the right adrenal gland is reported in a 49-year-old male who presented with progressive flank pain. This is the second case in the English language literature and the first to have documentation of malignant behavior. The tumor measured 11 cm in diameter and showed marked necrosis with prominent mitotic activity (average 15 per 10 high-power fields). Smooth muscle differentiation was apparent ultrastructurally and confirmed by positive immunostaining for muscle-specific and alpha-smooth muscle actin. Bony metastases developed; following palliative treatment with radiation and chemotherapy, the patient is alive with tumor 9 months later. Origin from smooth muscle associated with the central adrenal vein or its tributaries is proposed.

Adrenal Gland Neoplasms

Cytometry and morphometry of malignant fibrous histiocytoma of the extremities. Prediction of metastasis and mortality.

Flow cytometry and nuclear morphometry were compared with traditional pathologic grading techniques for predicting the course of malignant fibrous histiocytoma of the extremities. Clinical, pathologic, and flow/morphometric variables from 53 cases were tested by Cox regression for prediction of distant recurrence and mortality. Tumor grading based on extent of tumor necrosis was a significant predictor for both disease-free survival (p = .014) and overall survival (p = .003). The fraction of nuclei in the S + G2M segment of DNA histograms was significant for disease-free survival (p = .007), and remained significant (p = .033) in a joint Cox model with necrosis-based grade (p = .004 for the bivariate model). Relative risk for recurrence varied nearly 10x between the 10th and 90th percentiles of grade and (S + G2M)1/2. Overall survival was predicted by a nuclear shape feature termed "R" (p = .000008), the casewise difference (residual) between expected and observed nuclear perimeter as a function of average Feret diameter. In a bivariate Cox model, relative risk of mortality varied 35x between the 10th and 90th percentiles of grade and R. Cytometric and morphometric data contain information about recurrence-free and overall survival beyond that available from more usual clinical and pathologic features. It seems likely that nuclear morphometry, in particular, will prove to be a useful aid for estimating the prognosis of patients with malignant fibrous histiocytoma of the extremities.

DNA, Neoplasm