Harry L. Evans

Liposarcoma: a study of 55 cases with a reassessment of its classification.

Fifty-five cases of liposarcoma were reviewed. The cases were classified on the basis of histopathologic findings as follows: myxoid, 29 cases; well-differentiated, 11 cases; dedifferentiated, eight cases; pleomorphic, four cases; combined well-differentiated and pleomorphic, two cases; and unclassified, one case. The term “dedifferentiated liposarcoma” is proposed for tumors containing distinct areas of well-differentiated liposarcoma and cellular nonlipogenic spindle-cell or plemorphic sarcoma. All patients except one were adults. Myxoid and plemorphic liposarcoma predominantly involved the thigh, while well-differentiated, dedifferentiated, and combined well-differentiated-plemorphic liposarcoma showed a marked preference for the retroperitoneum. The survival of patients with pleomorphic and dedifferentiated liposarcoma was significantly poorer than that of those with the myxoid and well-differentiated varieties. There was a marked tendency toward local recurrence in all types. Recurrent myxoid and pleomorphic liposarcomas were often controlled by additional therapy, whereas recurrences of other types usually were not; this difference was considered largely a result of locational factors. Metastasis was observed in 10 cases of myxoid liposarcoma, three of plemorphic liposarcoma, and two of dedifferentiated liposarcoma. In myxoid liposarcoma, initial treatment consisting of excision followed by radiation resulted in a significantly reduced rate of local recurrence as compared with excision alone, and more extensive as compared with excision alone, and more extensive hypercellular zones were associated with increased metastatic potential. “Round cell” liposarcoma was not found to be an independent type; all cases for which this designation might have been considered could be better classified as myxoid liposarcoma with cellular areas (usually) or plemorphic liposarcoma with rounded cells (rarely). The resemblance of portions of some dedifferentiated and plemorphi liposarcomas to “malignat fibrous histocytoma” was sufficient to deny the specificity of the histologic pattern of the latter.

Extraskeletal osteosarcoma : a clinicopathologic review of 26 cases

The clinical records and histopathologic features in 26 cases of extraskeletal osteosarcoma (ESOS) diagnosed at M. D. Anderson Cancer Center (Houston) between 1950 and 1987 were reviewed. Presentation was usually that of an enlarging soft tissue mass. The thigh (11 cases), upper extremity/shoulder girdle (three cases), and retroperitoneum (three cases) were the most common anatomic sites. Tumor size ranged from 2.5 to 30 cm. The predominant histologic pattern was osteoblastic in four cases, chondroblastic in two, fibroblastic or pleomorphic malignant fibrous histiocytoma (MFH)‐like in four, giant cell type MFH‐like in one, and small cell in one. Various mixtures of these patterns were seen in the remaining 14 tumors. The telangiectatic pattern was not seen as the predominant component in any primary tumor but was observed as a minor component. Thirteen tumors recurred locally and 16 metastasized; five patients had distant metastases at presentation. The lungs, bone, and soft tissue were the most frequent metastatic sites. Sixteen patients died of disease at 2 to 54 months, one patient died of unrelated causes at 61 months, seven patients were alive with no evidence of disease (NED) at 30 to 122 months, and two patients were alive with disease at 28 and 54 months, respectively. Tumor size (< 5 cm versus < 5 cm) was the main prognostic factor; all patients alive with NED for whom accurate tumor measurements were available (six of seven) had neoplasms measuring < 5 cm that were amenable to complete surgical excision. Histologic pattern and other clinicopathologic features did not significantly affect outcome.

Leiomyosarcoma of soft tissue: A clinicopathologic study

The case records and histologic material of 28 patients with leiomyosarcoma of soft tissue treated at M. D. Anderson Hospital between 1948 and 1975 were reviewed. These included patients in whom the tumors arose in the retroperitoneum (16), major blood vessels (3), and other soft‐tissue sites, collectively designated peripheral soft tissue (9). Follow‐up was complete in all cases. A striking preponderance of female patients was observed in the group with retroperitoneal tumors, in contrast to the male preponderance in the vascular and peripheral soft tissue tumor groups. Tumor size and location were the major prognostic factors; large size (≥5 cm) and retroperitoneal origin were associated with a much poorer clinical outcome than smaller size (<5 cm) and origin elsewhere than the retroperitoneum. These factors were related, since all retroperitoneal tumors were large. Microscopic appearance did not correlate significantly with clinical course. The clinical approach to these tumors is discussed in light of the findings of this study.

Sarcomatoid squamous cell carcinoma of the mucous membranes of the head and neck: a clinicopathologic study of 20 cases.

Twenty cases of head and neck mucosal squamous cell carcinoma containing a prominent sarcomatoid element were reviewed with special attention to possible prognostic factors. Patients whose tumors invaded muscle, minor salivary or accessory respiratory glands, or bone had very poor survival rates, whereas those whose tumors were superficial and did not extend into any of these structures had excellent survival rates. A history of irradiation to the tumor site and tumor location in the oral cavity rather than the larynx, pharynx, nasal cavity, or nasal‐associated structures were associated with invasiveness and thus with poorer survival. Aside from invasion, histologic features and gross configuration were not found to be of significant prognostic importance.

Desmoid Tumors: A 20-Year radiotherapy experience☆☆☆

From 1964 through 1984, 45 patients were referred for radiation therapy for desmoid tumor. Fourteen patients had inoperable lesions, or gross residual disease after incomplete resection. Thirty-one patients received postoperative XRT for positive margins or concern about the adequacy of the margin. The minimum follow-up was 2 years, maximum 22 years, median 7.6 years. No patient was lost to follow-up. The primary site was head and neck in 5, upper extremity in 10, chest wall and back in 8, abdomen 2, pelvis 4, and lower extremity 16. All patients were treated with megavoltage radiation therapy using shrinking field techniques. Large fields received a median dose of 50 Gy in 25 fractions. Boost fields were used in the majority of patients to deliver an additional dose of 7 to 27 Gy. The range of total doses was 50 to 76.2 Gy. Three patients received a boost with neutrons. Analysis of patients with inoperable or gross residual showed tumor control in 10 of 14 with a median follow-up of 9.4 years. Resolution of gross disease occurred at a range of 1/2 to 64.3 months with a median of 9 months. There was no evidence of a higher probability of ultimate control at higher doses. Tumor control was equal for men and women. The ten patients with local control had doses from 50 to 76.2 Gy whereas the four patients with in field failures had tumor doses of 57 to 66.4 Gy. There was no difference in median dose for patients with local control (60.3 Gy) versus those with tumor recurrence (60 Gy). For subclinical disease, 31 patients receiving postoperative or preoperative XRT had a 77 percent probability of local control in spite of the history of multiple tumor recurrences; local control was achieved in 8 of 9 with negative or uncertain margins and 16 of 22 with positive margins. An analysis of local control as a function of the number of operations revealed that patients referred for adjuvant radiotherapy with no more than two operative procedures had an 88 percent probability of local control, versus 66 percent for more than two operative procedures. All grade 3 complications (defined as requiring surgical intervention or prolonged hospitalization) occurred with doses above 60 Gy. Management of recurrences was successful in 8 of the 11 patients and no patient has died of tumor.(ABSTRACT TRUNCATED AT 400 WORDS)

Treatment of patients with isolated axillary nodal metastases from an occult primary carcinoma consistent with breast origin.

The records of 42 patients who had axillary metastases compatible with a clinically occult breast primary were reviewed. Forty patients had mammography performed as part of their evaluations. Mastectomy yielded the primary tumor in one of 13 patients; biopsy yielded positive results in one of five. Among the 29 patients who did not undergo mastectomy, 16 received breast irradiation, and 13 were simply observed for signs of the primary tumor. For the patients who did not undergo mastectomy, the 5‐year actuarial risk for appearance of a primary was 17% in the irradiated group versus 57% in the nonirradiated group (P = 0.06). Patterns of failure are correlated with stage and local and systemic therapy. The results affirm our belief that patients with axillary metastases histologically consistent with breast tumor should be treated identically to patients with similar nodal stages and proven breast primaries.

Dermatofibrosarcoma protuberans. A clinicopathologic review with emphasis on fibrosarcomatous areas.

We reviewed 75 cases of dermatofibrosarcoma protuberans (DFSP) from the University of Texas M.D. Anderson Cancer Center. All accessions were examined for areas of giant cell fibroblastoma (GCF), but none was found. The 30 cases having a minimum of 5 years follow-up were studied in more detail. The histologic findings were typical of DFSP in 24 cases, whereas in six cases discrete areas with a fascicular or “herringbone” growth pattern, considered to represent fibrosarcomatous change (DFSPFS), were evident. The mitotic rate was usually but not always higher in fibrosarcomatous areas, and occasional examples of typical DFSP demonstrated relatively numerous mitotic figures (up to 35 per 10 high-power fields). Other histologic findings of interest were the presence of melanin in two cases of DFSP and the focal presence of a distinctive type of multinucleated giant cell similar to those seen in GCF in six cases. Patients with DFSP-FS differed from those with DFSP in that they had a higher median age (56 years vs. 37 years). Tumor location was similar in both groups, with the trunk being the most common site. No significant difference in either the rate of local recurrence or the interval until recurrence between DFSP and DFSP-FS was evident; the only factor strongly related to local recurrence was adequacy of surgical margins. However, the only two patients who died of tumor, including the sole patient with distant metastasis, had DFSP-FS. We conclude that DFSP-FS deserves recognition as a variant of DFSP.

Intraparenchymal nevus cell aggregates in lymph nodes: A possible diagnostic pitfall with malignant melanoma and carcinoma

&NA; It is well documented that nevus cells can be found within the fibrous capsule and trabeculae of lymph nodes; however, it is less well known that nevus cells can also be found in the lymph node parenchyma. We report the findings in 13 cases of nevus cell aggregates located within the cortical and/or medullary parenchyma of lymph nodes. Seven of the 13 patients had a primary diagnosis of melanoma, three had no known malignancy, one had breast carcinoma, one had adnexal carcinoma of the skin, and one had squamous cell carcinoma of the tonsil. Of the seven patients with melanoma, four had axillary lymph node dissections and three had inguinal lymph node dissections. The patient with adnexal carcinoma had metastatic carcinoma in 14 of 20 lymph nodes that had been dissected; one of them also had intraparenchymal nevus cells. The patient with squamous cell carcinoma of the tonsil had an intraparenchymal nevus cell aggregate in one of the 21 dissected lymph nodes; all 21 were negative for carcinoma. Nests of intraparenchymal nevus cells ranged from clusters of only a few cells up to 2.1‐mm aggregates. No mitotic figures, prominent nucleoli, or lymphatic–vascular invasion were detected in any of the melanocytic aggregates. The melanocytic cells of the nevus cell aggregates expressed S‐100 protein and/or MART‐1 but not gp100 protein (HMB‐45). Less than 1% of the nevus cells expressed Ki‐67. The purpose of this study was to draw attention to the finding of nevus cells in the parenchyma of lymph nodes and to alert pathologists to this as a potential diagnostic pitfall, especially in patients with concurrent melanoma or carcinoma. Awareness that nevus cells can be present in nodal parenchyma, analysis of their morphologic features (including comparison with any previous or existing melanoma or carcinoma), and immunophenotyping will help pathologists to establish the correct diagnosis in most instances.

Polymorphous low-grade adenocarcinoma: a study of 40 cases with long-term follow up and an evaluation of the importance of papillary areas.

Forty cases of polymorphous low-grade adenocarcinoma with a minimum of 10 years of follow up were reviewed. The patients included 13 men and 27 women age 22 to 71 years (median age, 54 years); 30 were white and 10 were black. The tumors were all intraoral, and the palate was the most common site (n = 24). Histologically, the neoplasms were characterized by nonencapsulated, infiltrative borders; bland, regular nuclei; and highly variable growth patterns, including tubular, solid, papillary, microcystic, cribriform (with true lumens), pseudoadenoid cystic (without true lumens), fascicular, single file, and strand-like. Papillary areas of more than focal extent were present in 17 cases, but these cases were otherwise similar to the remainder and were considered to form part of the spectrum of polymorphous low-grade adenocarcinoma. Thirteen patients had local recurrence, which was not controlled by subsequent treatment in six; six patients had cervical lymph node metastasis; three patients had distant metastasis; and five patients died of or with tumor after prolonged periods. There was a statistically significant relationship between more than focal papillary growth and cervical lymph node metastasis, and between positive or unknown surgical margins and local recurrence (although not uncontrolled local recurrence); however, these were the only independent statistically significant correlations found between any clinical or pathologic parameter and any aspect of tumor behavior or patient survival.

Malignant melanoma of soft parts (clear cell sarcoma). A study of 17 cases, with emphasis on prognostic factors

Seventeen cases of malignant melanoma of soft parts (clear cell sarcoma) are reported. The patients ranged from 9 to 70 years of age, but 13 were between 10 and 40 years of age. There were eight male patients and nine female patients. The most common tumor location (seven patients) was the foot, followed by the area around the knee (four patients). The usual histologic pattern was that of variably sized nests of uniform plump spindle cells with clear to pale cytoplasm separated by fine to coarse fibrous septa; however, variants with a substantial proportion of epithelioid cells, moderate to marked nuclear pleomorphism, predominantly diffuse growth, or a microcystic pattern were seen. Patient survival was relatively poor overall (median, 49 months; ten deaths due to tumor) and was determined mainly by distant metastasis (11 patients). Both survival and distant metastasis were correlated with tumor size (P < 0.01 for patients with tumors ≧ 5 cm versus < 5 cm). Other clinical and pathologic factors, including patient age, sex, and race, tumor location, duration of symptoms, initial therapy, mitotic rate, tumor necrosis, proportion of epithelioid cells, and nuclear pleomorphism had no significant relation to survival or distant metastasis when tumor size was taken into account. Local recurrence and regional lymph node metastasis each occurred in four patients.