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Biomedical subjects

B M Shmookler

Publications and source records attributed to B M Shmookler.

49 records · Page 3Linked to original sources

Aggressive (malignant?) epithelial odontogenic ghost cell tumor.

Although the calcifying odontogenic cyst (COC) is a well-established pathologic entity, it has been recognized that there is an odontogenic neoplasm with histologic features similar to the COC that has often been diagnosed as COC. Investigators have applied several terms to this neoplasm, but we prefer the term epithelial odontogenic ghost cell tumor (EOGCT). No reports have appeared previously in the literature describing either carcinoma arising in a COC or malignant EOGCT. Three cases are reported here with aggressive growth and histologic features strongly implying a malignant potential, although none is known to have metastasized. Two occurred in the maxilla and one in the mandible. One was discovered 9 years following removal of a typical COC, and another recurred rapidly after excision.

Adolescent↗

Superficial epithelioid sarcoma: a clinical and histologic stimulant of benign cutaneous disease.

Epithelioid sarcoma, a distinctive clinicopathologic entity, typically arises in the deep soft tissues of the upper and lower extremities. Infrequently the tumor originates in the subcutis or dermis, whereby it may be confused both clinically and microscopically with a granulomatous or benign ulcerative process. A case of superficial epithelioid sarcoma, initially diagnosed as an atypical mycobacterial infection, is presented, and its histologic distinction from granulomatous and benign fibrohistiocytic lesions is discussed.

Adult↗

Estrogen receptor protein in bone and soft tissue tumors.

Thirty-three histologically diverse bone and soft tissue tumors were analyzed biochemically for the presence of estrogen receptor protein (ERP) and progesterone receptor by means of a conventional, commercially available, steroid-binding assay (dextran-coated charcoal method) on fresh frozen tissue. These results were compared with analysis of ERP by using a specific monoclonal antibody both in an enzyme immunoassay and on frozen tissue sections by using immunohistochemical procedures. Frozen tissue sections were also examined for the presence of estrogen and progesterone receptors using fluorescein-labeled steroids. Six of the 33 tumors (18%) contained low levels of ERP ranging from 19 to 73 fmol/mg as determined by the dextran-coated charcoal method. The remaining 27 cases contained no (less than 10 fmol/mg) ERP. The ERP-positive group included a fibromatosis, leiomyosarcoma, liposarcoma (2 cases), neural sarcoma, and a synovial sarcoma. Four were high grades sarcomas, and two were low grade sarcomas. There was excellent agreement between the ERP levels determined by the dextran coated charcoal method and those determined by enzyme immunoassay. ERP could not be demonstrated immunohistochemically on frozen tissue sections of the tumors even though it could be demonstrated in breast carcinomas serving as positive controls. The failure of the immunohistochemical technique may be related to the low levels of ERP in these tumors and the difficulty of detecting antigen at threshold levels. Cytochemical localization of receptor protein employing fluoresceinated steroids did not correlate with cytosolic ERP as determined by enzyme immunoassay or the dextran coated charcoal method. Moreover, the high level of background fluorescence gave rise to a significant amount of intraobserver and interobserver variation. Although the clinical significance of ERP protein in mesenchymal tumors is still uncertain, the present findings, coupled with various clinical observations suggesting hormonal dependency of some mesenchymal tumors, indicate that investigation of a larger group of patients amenable to statistical analysis is warranted.

Adolescent↗

Liposarcoma occurring in children. An analysis of 17 cases and review of the literature.

Liposarcoma, one of the most common malignant mesenchymal neoplasms affecting adults, has rarely been documented to arise in children. Therefore, in this retrospective study, the clinicopathologic features of 17 liposarcomas retrieved from the files of the Armed Forces Institute of Pathology (AFIP) which occurred in patients between the ages of eight months and 15 years were analyzed. In contrast to the slight male prevalence verified in most studies of adult liposarcoma, this group of 17 cases disclosed a distinct female majority (65%). The tumor had a predilection for the lower extremity (41%), particularly the thigh; the remaining examples, which included cases from the neck, back, axilla, buttock, and forearm, reflected a seemingly random anatomic distribution. The retroperitoneum, a very common primary location for adult liposarcoma, accounted for only one example in the group. The histologic variants of liposarcoma coincided with the types delineated in most generally accepted classifications employed for liposarcoma in adults, but their distribution varied considerably from that found in most extensive series of adult liposarcomas. Specifically, there were 13 myxoid liposarcomas, two well-differentiated liposarcomas, and one each of the round cell and mixed variants. Follow-up information, obtained for 15 cases, revealed local recurrence in three patients, one of whom eventually died as a result of direct tumor extension into the pleural cavity.

Adolescent↗

Retroperitoneal leiomyosarcoma. A clinicopathologic analysis of 36 cases.

Thirty-six cases of retroperitoneal leiomyosarcoma form the basis for this retrospective clinicopathologic study. This group comprised 24 females (67%) and 12 males (33%), whose ages ranged from 12 to 94 years (median, 60 years.). The clinical presentation, invariably nonspecific, consisted of pain or weight loss, typically associated with a palpable abdominal mass. Of the 30 patients with follow-up data, 23 (77%) died of retroperitoneal leiomyosarcoma. Grossly, the bulky often multinodular tumors, which ranged from 7.5 to 35 cm in maximal dimension (median, 12.8 cm), varied from firm to soft. In addition to the classical microscopic picture of leiomyosarcoma as manifested by interlacing fascicles of slender eosinophilic cells, other less frequently encountered, morphologic variations of malignant smooth-muscle tumors were also observed. Although absolute minimal criteria for a malignant tumor diagnosis could not be established, the findings suggest that a retroperitoneal smooth-muscle tumor that measures at least 7.5 cm in greatest dimension and that has as few as 1 mitosis/10 HPF is capable of metastasis.

Adolescent↗

Orofacial synovial sarcoma: a clinicopathologic study of 11 new cases and review of the literature.

Synovial sarcoma arises most commonly in the lower extremity, particularly in the region of the lower thigh and knee. Yet the occurrence of thus mesenchymal neoplasm in the head and neck area has been convincingly documented, albeit confined almost exclusively to cervical and parapharyngeal sites. Therefore, in view of its rarity in the head and neck, we analyzed a group of 11 synovial sarcomas arising in the orofacial region. The series comprised nine men (82%) and two women (18%). In common with synovial sarcoma at more conventional sites, this is a disease of young adults: ages ranged from 16-49 years (median, 34 years). Topographically, two subsets were delineated, a more common facial group with eight cases (four cheek, two parotid region, one infraorbital, one submental), all arising as gradually enlarging, usually nontender, solitary tumors; and three intraoral ones (two tonsillar, one lingual), two of which were polypoid and one was an exophytic tonsillar mass which presented with hemoptysis and stridor. Follow-up data, obtained for nine patients (range, 1.3-15.0 years), disclosed three (33%) tumor-related deaths, all belonging to the facial group. Treatment, difficult to significantly correlate with survival in this small series, varied from surgical excision alone to a multimodality approach including both irradiation and chemotherapy. Histologically, all the neoplasms revealed characteristic biphasic features, predominantly fibrosarcomatous in one, but, more typically, showing epithelial clefts and/or pseudoacinar spaces in the others. Differential diagnosis, depending on the proportion of the biphasic components, ranged from spindle cell mesenchymal neoplasms to various adenocarcinomas, including those arising in mixed tumors of salivary gland.

Adolescent↗

Retroperitoneal synovial sarcoma. A report of four cases.

Synovial sarcoma most commonly affects the extremities, especially the lower thigh and knee region; other primary sites, in contrast, have been only infrequently reported. Therefore, we undertook this study of four synovial sarcomas arising in the retroperitoneum. This group, whose ages ranged from 17-57 years (mean, 36 years), comprised three males and one female. The nonspecific clinical presentations consisted of pain and/or a mass; diverse preoperative radiographic procedures merely served to reinforce the impression of a retroperitoneal mass. Histologically, all four examples revealed a typical biphasic pattern. Two cases showed a predominant spindle cell component; two others disclosed a more even distribution of the spindle-cell and epithelioid-cell elements, one of these containing, in addition, broad areas of stromal calcification. Follow-up data, obtained for two patients, indicated that both died as a direct result of peritoneal sarcomatosis. The differential diagnosis of selected spindle-cell and biphasic neoplasms known to arise in the retroperitoneum will be discussed.

Adolescent↗

Pleomorphic lipoma: a benign tumor simulating liposarcoma. A clinicopathologic analysis of 48 cases.

A clinicopathologic study of 48 cases of pleomorphic lipoma from the files of the Armed Forces Institute of Pathology reveals that this tumor occurs principally in males (83%) in the fifth to seventh decades (mean 57 years) and shows a predilection for the posterior neck, shoulder, and back. Typically, the lesion appears as a painless, circumscribed subcutaneous mass that, on gross examination, resembles an ordinary lipoma. However, microscopically, in contrast to the uniform appearance of the mature adipose tissue cells of an ordinary lipoma, this neoplasm is characterized by an intimate admixture of variably-sized fat cells, and bizarre, pleomorphic, multinucleated giant cells. Many of the latter cells show a distinctive floret-like arrangement of the nuclei and are associated with interlacing bundles of dense birefringent collagen. Despite this pleomorphic picture, which not infrequently leads to a misdiagnosis of liposarcoma, follow-up data obtained for 34 patients (median follow-up period of three years) establish the invariably benign clinical behavior of this unusual tumor.

Adult↗

Primary sarcoma of the pulmonary trunk and/or right or left main pulmonary artery--a rare cause of obstruction to right ventricular outflow. Report on two patients and analysis of 35 previously described patients.

Clinical and morphologic observations are described in two women with primary sarcoma of the pulmonary trunk, and observations in 35 previously described patients with primary sarcoma involving a major extrapulmonary pulmonary artery are summarized. The neoplasm produces symptoms by causing obstruction to right ventricular outflow or by dislodging tumor fragments to the smaller intrapulmonary pulmonary arteries with or without pulmonary infarction. The sarcoma nearly always arises from the pulmonary trunk to which it is firmly attached. Although it grows to a large size within the lumen, it infrequently, despite its highly malignant histologic pattern, extends through the wall of the pulmonary trunk or metastasizes outside the pulmonary circulation. It may mimic a variety of more common disorders. Diagnosis can be achieved by angiography and treatment starts with total excision.

Adult↗

Peritoneal carcinomatosis from an unknown primary site. Management of 15 patients.

AIMS AND BACKGROUND: Peritoneal carcinomatosis from an unknown primary site is a rare and ill-defined entity. This work attempts to identify clinical and pathological features of patients with this disease and report the results of an aggressive combined treatment modality. METHODS: Retrospective analysis was performed of medical records of 15 patients with peritoneal carcinomatosis with no primary site identified at a single institution between 1989 and 2000. A primary gastrointestinal cancer was ruled out after a thorough endoscopic and radiologic work-up and complete exploratory surgery. RESULTS: Four women and 11 men were identified; the average age was 49 years. All patients had cytoreductive surgery with peritonectomies; 4 patients underwent a second-look operation. Perioperative intraperitoneal chemotherapy was given to 10 of the 15 patients, and 9 patients received post-cytoreduction chemotherapy given intraperitoneally (1), systemically (7) or both intraperitoneally and systemically (1). Overall median survival from diagnosis was 19.0 months; 1 patient is alive with disease at 21 months; and 3 patients are disease-free at 17, 38, and 60 months from diagnosis. Significant positive predictive factors for survival were a small volume of ascites (P = 0.02), a large number of peritonectomies performed (P = 0.001), second-look cytoreduction (P = 0.003), perioperative intraperitoneal chemotherapy (P = 0.008) and postoperative chemotherapy (P= 0.01), either intraperitoneal or systemic. CONCLUSIONS: Peritoneal carcinomatosis from an unknown primary site is a rare subset of primary peritoneal malignancy. Aggressive treatment may provide prolonged palliation with occasional long-term survival.

Adult↗