Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.

Histopathologic material from 1,782 patients registered in the Intergroup Rhabdomyosarcoma Study Committee (IRS)-I and -II were reviewed by the IRS Pathology Committee in order to provide a uniform approach to classification and correlate patient survival with tumor type. Categories considered eligible were the four types of rhabdomyosarcoma (RMS) (criteria of Horn and Enterline), extraosseous Ewing's tumor (EOE), and a group of somewhat variable undifferentiated sarcomas designated small round cell sarcoma, type indeterminate (STI). Tumors that were clearly sarcomas but were unclassifiable also were included (NOS). The committee diagnoses were embryonal (Emb) RMS in 877 (54%), alveolar (Alv) RMS in 343 (21%), botryoid (Botr) RMS in 88 (5%), pleomorphic (Pleo) RMS in 11 (1%), STI in 135 (8%), and EOE in 84 (5%). One in nine were mixtures of types, eg, Emb and Alv. Five percent of the sarcomas could not be classified because of inadequate material. In general, there was close agreement (94%) between the review committee and institutional pathologists in the diagnosis of RMS, but not in the specific types, particularly Alv RMS (41%) and STI (36%). This observation is important, since patients with Alv RMS and STI tumors had decreased survival compared with the other histologies. The prognosis varied by histology, with Botr having the best, Alv RMS and STI the worst, and Emb RMS and EOE an intermediate prognosis.

Primary extranodal soft tissue lymphoma of the extremities.

Since true primary lymphomas of soft tissues are extremely rare and there are few well-documented cases in the literature, we report eight cases to help define the clinical and pathologic features of this type of extranodal lymphoma. Among 7,000 malignant lymphomas seen at Mayo Clinic over a 10-year period, we found eight cases of Stage IAE extranodal malignant lymphoma that presented as a soft tissue mass in the upper or lower extremities. The tumors occurred in the following sites: thigh (n = 4), upper arm (n = 2), ankle (n = 1), and calf (n = 1). Two cases had low-grade histology: small lymphocytic lymphoma and follicular mixed small-cleaved and large-cell lymphoma. An intermediate-grade histology was present in two cases: diffuse mixed small- and large-cell lymphoma and diffuse large-cell (cleaved) lymphoma. Half of the patients had a high-grade lymphoma: diffuse large-cell (n = 3) and large-cell, immunoblastic (n = 1). The results of immunohistochemistry and electron microscopy in selected cases are discussed in the context of the differential diagnosis with other small, round-cell cancers. Four patients died of recurrent lymphoma, one at 7 months, two at 15 months, and one at 4 years. Three patients are alive without evidence of disease at 3, 8, and 13 years. One patient is alive and is receiving chemotherapy for locally recurrent lymphoma 6 months after diagnosis.

Comparison of needle biopsy with cytologic analysis for the evaluation of pleural effusion: analysis of 414 cases.

We retrospectively studied the results from thoracenteses and needle biopsies of the pleura performed in 414 patients with pleural effusions between 1973 and 1982. The final causes of effusion were malignant disease in 281 patients (67.9%) and nonmalignant disease in 133 (32.1%). The presence of pleural malignant disease was established by cytologic study in 162 patients (57.6%), by needle biopsy in 123 (43%), and by either cytologic analysis or biopsy in 182 (64.7%). In only 7.1% of the 281 patients with malignant pleural effusions did biopsy reveal malignant disease when the results of cytologic study were negative for malignant disease. Nearly half of the patients with lymphoma had lymphocytosis of the pleural fluid, but neither this finding nor the lymphocytic pleuritis noted on biopsy was diagnostic of lymphomatous involvement of the pleura. Among the patients with malignant mesothelioma, thoracotomy was necessary to confirm the diagnosis in 60.9%. In the patients with nonmalignant diseases, with the exception of six with tuberculous pleurisy, pleural biopsy was nondiagnostic even though the causes of pleural effusion were apparent from the clinical features. The causes of pleural effusion remained unknown in 82 patients (19.8%). Pleural biopsy failed to provide adequate tissue in 55 patients (13.3%). This study shows that cytologic analysis has a higher sensitivity (P less than 0.001) than needle biopsy for diagnosing malignant pleural effusions. The value of needle biopsy is limited in establishing the cause of pleural effusion that results from either malignant or nonmalignant disease, with the exception of tuberculous pleurisy.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurofibrosarcoma of skin and subcutaneous tissues.

In this report, we describe 13 cases of primary neurofibrosarcoma of the skin. The tumor presumably arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei; hypocellular areas with loose, myxoid stroma; and areas of organoid organization such as palisading, whorly, storiform, and tactile body-like formations. The S-100 stain is positive in about 60% of cases. In the current series, most tumors arose in deep dermis and were grade 2 malignant lesions with a moderate degree of cytologic atypia and 2 or fewer mitoses in 10 high-power fields. Three patients died of their malignant lesion. Only two tumors metastasized. Of the 10 patients who had local recurrence, 5 had multiple recurrent lesions. Neurofibrosarcoma should be considered in the differential diagnosis of malignant tumors of the skin. A complete surgical resection of the primary tumor with adequate margins of surrounding normal-appearing tissue is advised.

Incidence of malignant bone and joint tumors in Olmsted County, Minnesota, 1935 through 1981.

During the 47-year period 1935 through 1981, 30 cases of malignant bone and joint tumors (in 20 male and 10 female patients) were identified among Olmsted County, Minnesota, residents. The incidence rates per 100,000 population, age-adjusted to the 1970 US total white population, were 1.0 overall, 1.4 for male subjects, and 0.7 for female subjects. Mortality rates, likewise age-adjusted, were 0.7 overall and 1.1 and 0.3 for male and female subjects, respectively. These rates are similar to those from tumor registries, a finding that suggests that reporting of these tumors is relatively complete. The similarity of the incidence and the mortality rates is consistent with the high case-fatality rate. A comparison of the Olmsted County cases with Mayo Clinic referral cases provides some evidence for referral bias, as the referral patients were significantly younger and had significantly more high-grade tumors.

Intrathoracic lymphangioma.

Of 14 cases of intrathoracic lymphangioma, 4 occurred in the anterior, 5 in the superior, and 4 in the posterior mediastinum. One lesion was diffuse and associated with disappearing bone disease (Gorham's disease). The anterior mediastinal lymphangiomas occurred in adults and seemed to arise from the mediastinum. They could not be distinguished from other anterior mediastinal lesions on plain film roentgenograms. The lesions of the superior mediastinum were extensions from cystic hygromas of the neck and occurred primarily in children. Recurrence was common because complete resection was not possible. Computed tomography was helpful in detecting the cervical extension and cystic nature of these lesions. Although two of the four posterior compartment lesions were isolated lymphangiomas, the other two were part of a much more extensive and generalized lymphangiomatosis that included bone lesions and subdiaphragmatic extension. Both of these latter cases were complicated by chylothorax after attempted removal of the mediastinal lymphangioma. Computed tomography and lymphangiography were helpful in determining the extent of the disease and the cystic and lymphatic nature of the mass. The patient with Gorham's disease had extensive lymphangiomatosis of the thorax and recurrent chylothorax; surgical treatment was unsuccessful. Although lymphangioma is a benign tumor, its infiltrative tendency complicates its removal and contributes to its postoperative recurrence.

Nonendocrine cancer of the pancreas in patients under age forty years.

In an attempt to determine the natural history of ductal adenocarcinoma of the pancreas in patients under 40 years of age, we reviewed the surgical outcomes of all such patients seen at the Mayo Clinic from 1970 to 1985. Histologic sections were reviewed; islet cell tumors and cystadenocarcinomas were carefully excluded. Twenty-six patients were identified. Their mean age was 34 years, with only one patient less than 25 years old. Symptoms included primarily abdominal pain, weight loss, and jaundice. One third of patients had a recent or past history of pancreatic disease including pancreatitis, pseudocysts, benign cystadenoma, and choledochal cyst. The tumor was located in the head of the gland in 62% of patients. "Curative" resections were possible in only three patients (12%); the remaining patients underwent palliative bypass (38%), biopsy alone (42%), or a palliative resection (8%). The hospital mortality rate was 12%, with actual 1-, 2-, and 5-year survival rates of 19%, 8%, and 4%, respectively, with a median survival of 4 months. The only long-term survivor underwent biliary bypass at age 15 years for a large neoplasm in the head of the gland; despite biopsy-proved liver metastases at that time, she continues to do well 5 years later. Histologic review indicated this tumor to be a "solid and papillary neoplasm of the pancreas." Ductal adenocarcinoma of the pancreas in young patients is an aggressive tumor with a poor prognosis behaving much like ductal adenocarcinoma in older patients (greater than 40 years). In rare instances a more favorable outcome can be expected when a solid and papillary neoplasm is found.

Management of retroperitoneal sarcomas.

The records of 116 adult patients who underwent operative treatment for retroperitoneal sarcomas at the Mayo Clinic during the years 1963 to 1982 were reviewed. Clinical, pathologic, and treatment variables were analyzed for their influence on recurrence and death from disease. Leiomyosarcomas, liposarcomas, and malignant fibrous histiocytomas represented 93% of the tumors. The primary tumor was completely excised in 54% of patients. Recurrent tumor developed in 68% of patients (median time to recurrence, 1.3 years). Tumor fixation to adjacent structures (T3 tumor) or a high-grade tumor (G2-4) identified patients at increased risk for recurrent disease. Five-year and 10-year survival rates were 40% and 22%, respectively. Survival was significantly better for patients who had (1) complete surgical excision of their tumors, (2) low-grade tumors (G1), (3) tumors not fixed to adjacent retroperitoneal structures (T1 and T2 sarcomas), and (4) tumors without metastases when initially seen. Complete surgical excision offers patients with retroperitoneal sarcomas the best chance for long-term survival, but recurrent disease remains a vexing problem. The therapeutic challenges in the treatment of retroperitoneal sarcomas continue to be the development of therapy that will increase the rate of complete resection, decrease the rate of local recurrence, and enhance patient survival.

Intravesical condyloma acuminatum with progression to verrucous carcinoma.

Condyloma acuminatum is a common form of venereal disease. Most patients with condylomata acuminata suffer from only the local cosmetic and irritative effects of the lesions. Few patients have progression to aggressive, regionally distributed lesions that can be life-threatening. We describe a forty-three-year-old white woman who had a seventeen-year history of scleroderma with extravesical and intravesical condylomata acuminata. During two years of conservative management with transurethral excision, electrocoagulation, and intravesical chemotherapy, the disease progressed to involve the entire bladder and resulted in obstructive renal insufficiency that required anterior exenteration and urinary diversion. The natural history of the disease is described, and the relevant literature is reviewed.

Correlation of DNA ploidy, histopathology, stage and clinical outcome in gastric carcinoma.

The determination of nuclear DNA ploidy from paraffin-embedded specimens was performed by flow cytophotometry on 277 surgically resected primary gastric carcinomas to assess the relationship of various pathological findings and DNA content with survival. The preparation of samples was performed by a modification of Hedley's technique and the staining method of Vindelov. Eighty-nine (32%) carcinomas were DNA diploid, 69 (25%) were DNA tetraploid, and 119 (43%) were DNA aneuploid. DNA non-diploid patterns were significantly associated with macroscopic ulcerative appearance, location of the tumour in the proximal stomach, histological grade, and advanced stage of tumour. Patients with DNA non-diploid cancers, and specifically DNA aneuploid cancers, exhibited significantly poorer survival than patients with DNA diploid tumours. These data support the prognostic value of tumour DNA content in patients with resected gastric carcinoma.

Tumor size and axillary metastasis, a correlative occurrence in 1244 cases of breast cancer between 1980 and 1995.

A review of 1244 breast cancer cases from the Tumor Registry of Northwest Community Hospital between 1980 and 1995 was carried out to investigate the incidence of axillary metastasis. There were 442 patients (35.45%) with positive nodes. The small lesions were graded to ascertain if size and grade of small tumors can be used to predict axillary metastasis. One hundred and seventy-nine cases of < or = 1.0 cm were retrospectively reviewed by one pathologist. Tumors <0.4 cm had negative nodes. Those with nuclear and histologic grades of 1 had 3% positive nodes, the remainder had positive nodes ranging from 11% to 19%. Infiltrating duct cancers of nuclear grade 3, histologic grade 2, and positive nodes, showed a 40% mortality. Eighteen patients died in the 0.5-1.0 cm tumor size range, mostly of histologic grade 2 and nuclear grade 3. Nuclear and histologic grade 1 tumors with infiltrating duct cancers had negative nodes and showed a good prognosis. Based on this study, node dissection can be omitted in these patients and in those with tumors < or = 0.4 cm. For all other lesions, full axillary node dissection and detailed pathologic examination is still the gold standard for evaluating the axilla.

Soft-tissue sarcomas of the head and neck.

We reviewed the charts of 352 patients at the Mayo Clinic who were diagnosed and treated for primary soft-tissue sarcomas of the head and neck from 1962 to 1982. The age at diagnosis ranged from 6 weeks to 91 years; 28 percent were pediatric patients. Regional or distant metastases were present at the time of primary diagnosis in 8 percent of patients. Nonorbital rhabdomyosarcoma was the most common tumor type (17 percent), and neuroblastoma was the rarest (2 percent). In children, rhabdomyosarcomas were proportionally more common, as was overall involvement of the orbit. Surgical excision was the sole method of tumor control in 49 percent of patients; excision with adjuvant radiotherapy or chemotherapy was used in 33 percent. In those with localized disease, overall survival was 81 percent at 2 years, 68 percent at 5 years, and 60 percent at 10 years postoperatively. Patients with angiosarcoma and nonorbital rhabdomyosarcoma experienced the poorest survival rates.

Extra-abdominal desmoid tumors.

One hundred and ninety-four patients with extra-abdominal desmoid tumors, most of them in the extremities, have been treated at the Mayo Clinic. One hundred and thirty-two patients (68 per cent) experienced a recurrence at an average of 1.4 years after the first treatment. A greater tendency for recurrence was evident in female patients, in patients who were more than thirty years old, in certain anatomical locations (especially the foot and calf), and most importantly after treatment by intralesional or marginal excision. In view of the excellent prognosis for survival, we recommend wide local excision when anatomically feasible or marginal excision and postoperative radiation therapy when function of the extremity would be severely compromised if excision with wide margins were done. A recurrent lesion that does not appear to be growing should be followed until evidence of growth of the lesion precipitates a secondary wide excision.

Epithelioid sarcoma. An analysis of fifty-one cases.

Analysis of the cases of fifty-one consecutive patients who had an epithelioid sarcoma revealed the five-year rate of survival to be approximately 70 per cent and the ten-year rate, approximately 50 per cent. The five-year rate of survival was about 40 per cent for the male patients and about 80 per cent for the female patients. If the primary tumor was more than three centimeters in diameter or was deeply situated, the patients had a reduced life-span, as did the patients in whom the tumor was focally necrotic. More male than female patients had necrosis of the tumor, as seen on the pathological specimens. No significant difference in life-span was noted among the patients in whom the resection had a marginal, wide, or radical surgical margin. The data indicate that wide or radical resection should be done as soon as epithelioid sarcoma is diagnosed.

Primary embryonal carcinoma and choriocarcinoma of the mediastinum. A case report.

Primary germinal tumors of the mediastinum are very rare. We studied a 67-year-old man with a primary embryonal carcinoma and choriocarcinoma of the mediastinum. To our knowledge, this is the oldest patient described as having this lesion. The diagnosis of primary germinal tumor of the mediastinum should not be made unless the possibility of a primary testicular lesion has been eliminated by thorough clinical evaluation. The prognosis is very poor for patients with teratocarcinoma, embryonal carcinoma, or choriocarcinoma, despite aggressive therapy.

Cartilage- and bone-forming tumors of the soft tissues.

Benign and malignant cartilage- and bone-forming tumors arising in soft tissues are unusual. Hyaline cartilage tumors may easily be mistaken as malignant if the histologic criteria of intraosseous lesions are applied directly. Other chondroid- and bone-forming tumors also may be difficult to recognize. The correct pathologic diagnosis within this group of neoplasms requires close clinical correlation, as well as familiarity and careful interpretation of a broad histologic spectrum.

Hibernoma. A case report.

Hibernoma is a rare soft-tissue tumor clinically presenting as a firm, freely movable, nontender mass, most commonly in the interscapular region. It is slow growing and usually asymptomatic. Examination of the overlying skin may demonstrate evidence of hypervascularity. On gross examination, the tumor is encapsulated and highly vascular, with its color varying from tan to reddish brown. Microscopic examination is usually diagnostic, demonstrating the characteristic multivacuolated oval or polygonal cells. Because of its benign character, treatment consists of simple excision. This article describes a case of a patient with hibernoma.