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Paratesticular soft tissue tumors are uncommon neoplasms that may present significant challenges for the pathologist and clinician. This article reviews the incidence of these rare tumors, and on the common benign and malignant entities that occur in this location. Key points in differential diagnosis are emphasized. The grading, staging, and management of patients with paratesticular sarcomas is also discussed.
Four soft tissue tumors corresponding with the previously reported parachordoma are described. Three of the patients were men, and one was a woman, and their ages ranged from 14 to 53 years (mean age, 29). The tumors were located either superficially or within muscle, and, in one case, involved a tendon. Histologically, the tumors displayed whorls, nests, and pseudoglandular cords of uniform polygonal cells with eosinophilic, vacuolated cytoplasm, in a focally myxoid stroma. Transitions were seen between fascicles of ovoid-spindled cells, with scanty cytoplasm in a fibrous stroma, and, in one case, whorls of bland spindly cells were also present. Electron microscopy of one case showed cells with short interdigitating microvilli and ill-defined junctions. The principal cells in all cases were positive for S100 protein, Leu-7, keratin (CAM5.2), and epithelial membrane antigen (EMA). All tumors were negative with antibody AE1 and with antibodies to cytokeratins CK7 and CK19. No tumor displayed immunoreactivity for carcinoembryonic antigen (CEA), muscle specific actin (MSA), smooth muscle actin (SMA), desmin, glial fibrillary acid protein (GFAP), CD31, or CD34. Parachordoma appears to be an entity with clinical and pathological differences from chordoma, which has a different cytokeratin profile, behaves in a more aggressive fashion, and can dedifferentiate. The differential diagnosis includes myxoid chondrosarcoma, myoepithelial cell tumor, ossifying fibromyxoid tumor, and chondroid lipoma.
Data regarding DNA content parameters in soft tissue sarcoma is limited. Development and optimization of tissue specific preparative techniques for DNA flow cytometry was undertaken prior to routine DNA content analysis of soft tissue neoplasms; 154 soft tissue tumors were studied. Dissociation dependent differences in cellular yields, viabilities, maintenance of DNA aneuploid populations, coefficients of variation, and DNA index supported the need for these developmental studies. Fifty-six of eighty-nine patients had DNA aneuploid soft tissue sarcomas. A relationship between DNA aneuploidy and grade was seen in this series with 38% with low grade, 59% with moderate grade, and 69% with high grade tumors demonstrating DNA aneuploid populations (P < 0.005). The mean S-phase fraction for DNA diploid and aneuploid sarcomas was 7.2% and 13.3%, respectively (P < 0.001). When classified by histologic grade of the primary tumor, a greater percentage of metastatic lesions were DNA aneuploid (4 of 7 grade 2 lesions, and 15 of 16 grade 3 lesions). Decreases in cellular yields and rate of DNA aneuploidy were observed in a subgroup of patients with localized high grade sarcoma treated preoperatively, as compared to patients treated with initial surgery. Prospective correlation of DNA content parameters to prognosis and response to cytotoxic therapy are now possible and are ongoing.
Chronic soft-tissue haematomas are infrequently reported in the literature. We present a case of a slowly expanding post-traumatic chronic soft-tissue haematoma located anterior to the knee and leg, occurring in a region previously unreported. Both clinically and radiologically, it was impossible to distinguish the haematoma from an aggressive soft-tissue neoplasm. We review the literature and discuss the diagnostic problems related to this uncommon presentation.
Development of a lipoma within the subcutaneous tissues of a trapezius myocutaneous flap is very rare. This is more so when it occurs after surgery for a head and neck cancer. In the case report described, it masqueraded as tumour recurrence causing significant anxiety. To the best of our knowledge this is a previously unreported 'complication'.
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant neoplasm that has the potential for aggressive local growth and destruction if not treated appropriately. Although the storiform arrangement of spindle cells in DFSP is relatively characteristic, histologic patterns simulating other benign as well as malignant neoplasms such as dermatofibroma, neurofibroma, malignant fibrous histiocytoma, and atypical fibroxanthoma have been described. METHODS: We collected and analyzed six cases of probable DFSP in which a specific diagnosis could not be rendered due to the predominant neurofibromatous changes in the histologic sections. In an attempt to reach a definitive diagnosis, the clinical history and physical characteristics of the lesions were taken into account, and all cases were further evaluated using immunostaining for CD34 and S-100 protein. RESULTS: The average age of the patient was 56 years (range 21-80), and the male to female ratio was 1 : 1. The location of lesions included the scalp, neck, back, and abdomen. All cases displayed two distinct histological patterns: (i) a proliferation of spindle cells with wavy nuclei in a loose mucinous stroma suggesting neural differentiation and (ii) a proliferation of spindle cells which interweaved and filled the reticular dermis extending into the subcutis. The wide variety of clinical impressions and descriptions indicated that the diagnoses were not always straightforward, and clinical information did not always assist in the clinicopathologic correlation. All lesions stained positively for CD34; however, three of six cases also stained positively for S-100. The three cases which were CD34 positive and S-100 negative were likely DFSP, and this was the final diagnosis given. The three cases that were CD34 and S-100 positive did not allow for a straightforward diagnosis. CONCLUSIONS: DFSP may demonstrate areas with features more characteristic of a benign neural lesion, such as a neurofibroma, which can lead to underdiagnosis and subsequent failure to treat. Clinicians and pathologists should recognize this potential diagnostic pitfall and understand that equivocal clinical information, combined with non-specific immunohistochemical staining patterns, can further complicate the dilemma. In these situations, where DFSP is the likely diagnosis but definitive evidence cannot be obtained, full excision of the lesion should be recommended to avoid mistreatment of a potentially malignant lesion.
Epithelioid sarcoma (ES) is a rare soft tissue neoplasm that usually occurs on the upper extremity of adolescents and young adults. It rarely occurs during childhood. ES is a slowly growing tumor with a high propensity for recurrences and metastases. This neoplasm is likely to be confused with a variety of benign and malignant conditions. Recognition of ES is important. The treatment consists of wide excision. Misdiagnosis can lead to inappropriate treatment and local recurrences or metastases. We report the case of a 5-year-old girl with an ES on the right forefinger.
Neoplasms of the soft tissue range from benign, indolent tumors to some of the most aggressive malignancies known to man. Despite exhaustive studies detailing their occurrence elsewhere within the body, there has been relatively little detailing such neoplasms arising within the soft tissues of the foot. Due to the relative infrequency with which malignant soft-tissue tumors occur in the foot, large series pertaining to this location have been difficult to assemble. To better elucidate the relative frequency of various benign and malignant soft-tissue tumors of the foot, the clinicopathologic features of 401 such neoplasms are described. All cases were retrieved from the files of a major medical center which specializes in the treatment of cancer. Of the 401 cases identified, 149 were benign, while 252 cases were of a malignant nature. The clinicopathologic features are summarized and descriptive statistics are applied. Special focus is given to the discussion of the malignant and rare tumors within this series. Recent concepts regarding diagnosis, prognosis, and therapy are similarly discussed.
The computed tomographic scans (CT) of 84 patients with untreated soft-tissue neoplasms were studied, 75 with primary and nine with secondary lesions. Each scan was evaluated using several criteria: homogeneity and density, presence and type of calcification, presence of bony destruction, involvement of multiple muscle groups, definition of adjacent fat, border definition, and vessel or nerve involvement. CT demonstrated the lesion in all 84 patients and showed excellent anatomic detail in 64 of the 75 patients with primary neoplasms. CT did not differentiate vessel or nerve entrapment from neurovascular structures that were simply applied to the pseudocapsule of the tumor. Blurring of adjacent fat was an infrequent finding, but when it was present, the tumor was malignant. The CT findings were characteristic enough to suggest the histology of the neoplasm in only 13 lesions (nine lipomas, three hemangiomas, one neurofibroma). No malignant neoplasm had CT characteristics specific enough to differentiate it from any other malignant tumor. However, malignant neoplasms could be differentiated from benign neoplasms in 88% of the cases.
In order to evaluate the role of Computed Tomography (CT) in prediction of nature, staging and follow-up of soft-tissue tumors, the authors examined by CT 124 patients with soft tissue neoplasms who later underwent surgery (116 cases) or fine needle biopsy (8 cases). Comparison between CT and surgical or anatomical results showed that CT was able to correctly predict the benignancy or malignancy of the masses in 76% of cases but it was very seldom able to allow an histological prediction. On the contrary CT was found to be a very useful tool for pre-therapeutic staging and follow-up of the tumors, because it gave many diagnostic information which influenced therapeutic choices and strategies.
A project for the classification of malignant soft tissue tumors according to the TNM system is suggested for a wide discussion. The classification takes into account the effect of some leading factors (histological type of a tumor, its size, the state of regional lymph nodes, the degree of the adjacent tissues infiltration) on the prognosis of this affection. Tumors staging is done through combining various grades of T, N, M criteria.
Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.
The authors discuss various radiographic imaging techniques for pedal soft tissue lesions. These include xeroradiography, angiography, ultrasound, computed tomography, and magnetic resonance imaging. Illustrative cases of each modality, as well as an evaluation of advantages and disadvantages, are presented.
Granuloma inguinale has been reported to mimic squamous cell carcinoma, as well as sexually transmitted diseases. We describe a rare case of extragenital granuloma inguinale that mimicked a soft tissue neoplasm in an 18-year-old pregnant woman who presented with cervical and labial ulcers. An elective cesarean section was performed, at which time a retroperitoneal mass that involved the right ureter was found. At frozen section, the mass had a xanthogranulomatous appearance consistent with a soft-tissue neoplasm. Special stains of the permanent sections revealed the pathognomic features of granuloma inguinale.
Combination of fat suppression techniques with intravenous paramagnetic contrast administration is usually performed using high-field MR systems. We combined a modified three-point Dixon technique for fat suppression with gadolinium-DTPA administration in the investigation of soft tissue neoplasms at 0.5 T. Nineteen patients with 21 neoplasms (14 primary malignant, 2 metastatic, 5 benign tumors) were examined. Examination protocol included unenhanced SE T1, PD and T2-weighted images. After the intravenous administration of gadolinium-DTPA (0.1 mmol/kg), a modified three-point Dixon technique provided three image sets, i.e., conventional T1-weighted SE images, fat-suppressed T1-weighted images and water-suppressed T1-weighted images. Two observers evaluated the lesions for conspicuity and size in three image groups: unenhanced T1-, PD and T2-weighted images, enhanced conventional T1-weighted images, enhanced T1-weighted images with fat suppression. Ten lesions showed greater conspicuity on enhanced fat-suppressed images than on unenhanced T1-, PD and T2- and enhanced conventional T1-weighted images. In 16 cases lesions were more conspicuous on enhanced water images than on conventional enhanced T1 images. Ten tumors exhibited a larger size (difference exceeding 20%) on enhanced fat-suppressed images. The combination of gadolinium with the modified Dixon technique improves the demonstration of soft tissue neoplasms at 0.5 T and allows lesion size to be measured more accurately.
Immunohistochemistry is an extraordinarily powerful tool in the armamentarium of the diagnostic surgical pathologist. This article discusses some ways in which immunohistochemistry assists in the diagnosis of soft tissue neoplasms. Four common clinicopathologic scenarios in soft tissue pathology are presented and a practical approach to these rare neoplasms is discussed. The limitations of immunohistochemistry and possible future directions for the application of immunohistochemistry to sarcoma diagnosis are also reviewed.
We defined the immunocytochemical expression of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) in benign soft tissue neoplasms, fibromatoses, and sarcomas, together with the activity of gelatinase MMPs and TIMPs measured by zymography and reverse zymography in a subset of cases. The most strongly expressed MMP in all tumors was MMP-1, with weaker expression of MMP-10, MMP-11, and MMP-14 in most tumors. Nuclear expression of MMP-1, MMP-8, and MMP-13 was an unusual feature. TIMP-2 was expressed in all tumors, with stronger expression in fibromatoses than in sarcomas. Fibromatoses and high-grade sarcomas showed greater MMP-1 expression than other groups, and endothelial MMP-2 expression was more extensive in sarcomas. Differences in MMP and TIMP expression might be linked to the biologic behavior of soft tissue neoplasms. The activation of endothelial MMP-2 linked to widespread MMP-14 expression provides a mechanism for sarcomas to modulate their matrix and facilitate angiogenesis.
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