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PubMed1999

Tumors of the skin composed of large cells with abundant eosinophilic cytoplasm.

Tumors of the skin composed of large epithelioid cells displaying abundant smooth or granular eosinophilic cytoplasm are relatively rare, yet may constitute a source of diagnostic difficulties for pathologists. Lesions derived from various skin elements are represented in this group, including tumors originating from adnexal structures and connective tissue elements of the dermis. The large, pink-cell or epithelioid appearance of the tumor cells may be the result of attempts to recapitulate the phenotype of a cell line normally characterized by such morphological appearance, such as apocrine epithelium, or may result from secondary changes in the cytoplasm of the tumor cells, such as accumulation of autophagosomes in granular cell tumors. The characteristic pathological features of this group of tumors is the subject of this review, with special emphasis on their differential diagnosis and the morphological features that may be of help for practicing pathologists to arrive at a correct diagnosis.

Adenoma, Oxyphilic↗
PubMed2000

Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

Rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.

Adult↗
PubMed1996

Primary and metastatic malignant tumors in soft tissue of skin. Diagnostic, surgical, and prognostic considerations for the foot.

Benign and malignant soft-tissue tumors are those that arise from mesenchymal (and some neuroectodermal) nonosteogenic and nonchondrogenic cells. The various primary malignant tumors are sarcomas with invasive and metastatic potential and are the same types that arise in the deeper tissues. In addition to sarcomas that arise directly in the dermis or subcutis, these tissue areas may be invaded by sarcomas from the deeper structures and by carcinomas and sarcomas metastatic to the skin. Dermal or subcutaneous cancers may appear clinically nondescript but are nonetheless dangerous, requiring an appropriately considered biopsy for identification and subsequent planning of patient care.

Carcinoma↗
PubMed1997

Is primitive neuroectodermal tumor of the kidney a distinct entity?

BACKGROUND: Primitive neuroectodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectodermal origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton in adolescents and young adults. As a soft tissue neoplasm, PNET arising in the kidney has not been well described, with only three cases previously reported. METHODS: Four patients with PNET of the kidney were diagnosed and treated at St. Jude Children's Research Hospital. The authors reviewed the clinical, radiologic, and pathologic features and outcomes of these cases and of those previously described. RESULTS: The authors' patients were age 4-20 years. They presented with unilateral renal masses and metastatic disease in the lymph nodes (three patients), lungs (three patients), bone (two patients), liver (one patient), and bone marrow (one patient). Treatment included surgery, radiotherapy, and multiagent chemotherapy. Three of the patients died of progressive disease within 14 months of diagnosis. Features and outcomes were similar to those of the three previously reported cases. CONCLUSIONS: PNET of the kidney appears to be a distinct entity. Although rare, it must be included in the differential diagnosis of renal tumors in children and young adults. Patients usually present with advanced disease and show poor response to combined-modality therapy.

Adolescent↗
PubMed2002

[Cutaneous mesenchymal neoplasms versus mesenchymal neoplasms of subcutaneous and deep soft tissue. Similarities and differences].

As in subcutaneous and deep soft tissues, cutaneous mesenchymal neoplasms are also relatively rare and characterized by a clinicopathological heterogeneity. There are striking differences in regard to the occurrence of different lines of differentiation of mesenchymal neoplasms in superficial and deep locations, and some distinct entities arise exclusively in the dermis. Superficially located mesenchymal neoplasms are characterized by a number of specific morphological features in comparison to deeper located lesions. Clinically important, dermal sarcomas of some lines of differentiation are associated with a favorable clinical prognosis, independent of the histological grading, which is important to know for the correct treatment of patients. In the following review similarities and distinguishing clinicopathological features of superficially and deeply located mesenchymal tumors are discussed.

Diagnosis, Differential↗
PubMed2006

Prevalence, extension and characteristics of fluid-fluid levels in bone and soft tissue tumors.

The purpose of this study was to determine the prevalence, extension and signal characteristics of fluid-fluid levels in a large series of 700 bone and 700 soft tissue tumors. Out of a multi-institutional database, MRI of 700 consecutive patients with a bone tumor and MRI of 700 consecutive patients with a soft tissue neoplasm were retrospectively reviewed for the presence of fluid-fluid levels. Extension (single, multiple and proportion of the lesion occupied by fluid-fluid levels) and signal characteristics on magnetic resonance imaging of fluid-fluid levels were determined. In all patients, pathologic correlation was available. Of 700 patients with a bone tumor, 19 (10 male and 9 female; mean age, 29 years) presented with a fluid-fluid level (prevalence 2.7%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included aneurysmal bone cyst (ten cases), fibrous dysplasia (two cases), osteoblastoma (one case), simple bone cyst (one case), telangiectatic osteosarcoma (one case), "brown tumor" (one case), chondroblastoma (one case) and giant cell tumor (two cases). Of 700 patients with a soft tissue tumor, 20 (9 males and 11 females; mean age, 34 years) presented with a fluid-fluid level (prevalence 2.9%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included cavernous hemangioma (12 cases), synovial sarcoma (3 cases), angiosarcoma (1 case), aneurysmal bone cyst of soft tissue (1 case), myxofibrosarcoma (1 case) and high-grade sarcoma "not otherwise specified" (2 cases). In our series, the largest reported in the literature to the best of our knowledge, the presence of fluid-fluid levels is a rare finding with a prevalence of 2.7 and 2.9% in bone and soft tissue tumors, respectively. Fluid-fluid levels remain a non-specific finding and can occur in a wide range of bone and soft tissue tumors, both benign and malignant. Therefore, they cannot be considered diagnostic of any particular type of tumor, and the diagnosis should be made on the basis of other radiological and clinical findings.

Adolescent↗
PubMed1995

Solitary fibrous tumors of soft tissue. A clinicopathologic and immunohistochemical study of 12 cases.

We describe 12 cases of primary soft tissue neoplasms that showed the histologic and immunohistochemical features of solitary fibrous tumors of serosal surfaces (solitary fibrous mesothelioma, submesothelial fibroma). Nine patients were women and three were men whose ages ranged from 28 to 83 years. Seven lesions were located in the head and neck region, and the remainder were located in the back, buttock, perineum, and groin. The lesions measured from 1 to 6 cm in greatest diameter and presented grossly as well-circumscribed, unencapsulated, soft to rubbery tissue masses. Histologically they were characterized by a proliferation of spindle cells exhibiting a variety of growth patterns, including storiform, herringbone, neural with wavy nuclei, and hemangiopericytic admixed with areas of sclerosis. In two cases the lesions showed areas of increased cellularity with occasionally scattered mitotic figures. Three cases were located adjacent to a major salivary gland; in one, entrapment of normal salivary gland acini and ducts could be observed at the edges of the lesion. Immunohistochemical studies showed positive staining of the spindle cells with CD34 (anti-HPCA-1) and vimentin antibodies and negative staining with keratin, actin, desmin, S-100 protein, collagen type IV, and factor VIII related antigen. Follow-up from 6 months to 12 years has shown no evidence of recurrence or metastasis in any of our patients. Solitary fibrous tumors appear to represent ubiquitous mesenchymal neoplasms that may not be necessarily restricted to serosal surfaces. Identification of these lesions is of importance to avoid misdiagnosis with other more aggressive conditions in soft tissue locations.

Adult↗
PubMedDate not supplied

Infantile hemangiopericytoma--case report and literature review.

Infantile hemangiopericytoma is a rare soft tissue neoplasm of pericytic origin and is almost always benign, despite its worrisome pathologic features. We describe a 2-month-old male infant with a soft tissue mass on his right thigh. Histologically, the lesion showed a characteristic hemangiopericytoma-like vascular pattern, multilobulation, and moderate mitotic activity. These morphologic features were prediagnosed as infantile hemangiopericytoma. However, immunohistochemical and ultrastructural studies revealed a heterogeneous cellular composition, primarily pericytes and endothelial cells, similar to that observed in infantile myofibromatosis.

Diagnosis, Differential↗
PubMed1997

[Soft tissue sarcomas as a rare cause of pleural effusion].

Malignant disease is the cause of about 24% of all pleural effusions. They are caused mainly by lung and breast cancer. Three cases of pleural effusion caused by very rare neoplasm, soft tissues sarcoma are presented. In two of them the lesion found in the leg was observed for 4-14 month and not connected with the presence of pleural effusion. Difficulties in the histologic diagnosis of pleural sarcoma and of differentiating this tumour from mesothelioma are also presented.

Adult↗
PubMed1992

MR imaging of extraabdominal desmoids: differentiation from other neoplasms.

A retrospective review of MR images of 36 patients with histologically proved extraabdominal desmoids was done to define the MR characteristics of these tumors and to determine if MR could be used to differentiate desmoids from other benign and malignant soft-tissue neoplasms. The desmoids evaluated included eight primary and 30 recurrent lesions. Our study was conducted in parallel with another study in which the MR appearance of 95 benign and malignant soft-tissue masses was evaluated, and the MR images of these masses were compared with our findings. The four desmoids from that study are included in our data. The signal intensity of the tumor on T1- and T2-weighted images was graded relative to the intensities of muscle and fat. Homogeneity, margin, neurovascular and bone involvement, and fibrosis (low-signal regions within the tumor on both T1- and T2-weighted images) were evaluated. On MR imaging, the desmoids showed inhomogeneous signal (97%), poor margination (89%), neurovascular involvement (58%), and bone involvement (37%). Fibrosis was present in 88% of primary desmoids and 90% of recurrent ones, and intermediate signal (greater than that of muscle and less than that of fat) was present in 75% and 50% of these, respectively. Our results show that the MR features of desmoids have characteristics that are commonly found in malignant tumors (inhomogeneous signal, poor margination, and neurovascular involvement). MR features of desmoids that distinguish them from malignant neoplasms are the presence of fibrosis and intermediate signal in the regions of the tumor.

Adolescent↗
PubMed1987

Connective tissue nevus masquerading as a localized linear epidermal nevus.

A 22-year-old black female patient presented to the dermatology clinic with an eruption on her left arm. The eruption consisted of hyperpigmented grouped papules with a verrucous appearance. Because the lesions were soft on palpation, the eruption was diagnosed clinically as an atypical localized linear epidermal nevus. Examination of a biopsy specimen of the lesion, however, revealed marked fibroblastic proliferation and pronounced dermal fibrosis, along with epidermal changes of hyperkeratosis, focal hypergranulosis, and papillomatosis. These findings are consistent with connective tissue nevus with epidermal changes suggestive of epidermal nevus. Therefore a deep biopsy is strongly suggested to rule out the possibility of connective tissue nevus in suspected cases of atypical linear epidermal nevus.

Adult↗
PubMed2006

[Ganglioneuroma of the zygoma].

INTRODUCTION: Ganglioneuroma is an uncommon benign tumor that arises from the sympathetic nervous system accounting for less than 1% of all soft-tissue neoplasms. CASE REPORT: We report the case of a 22 year-old man who presented a ganglioneuroma localized in the zygoma. DISCUSSION: Ganglioneuroma generally develop in the abdomen or thorax soft tissue. The occurrence of ganglioneuroma in the bone is exceptional. To date less than ten cases have been reported, six of which involved the mandible. We discuss the possible causes of tumor development at this site.

Adult↗
PubMed1981

Immunohistochemical localization of actin: applications in surgical pathology.

The contractile protein actin was demonstrated in formalin-fixed and paraffin-embedded human tissue by the peroxidase-antiperoxidase method of Sternberger. Both types of muscular cells and some other cell types showed positive staining. In skeletal muscle, the staining was seen diffusely in the cytoplasm with accentuation of cross-striations (I-bands). The cytoplasm of smooth muscle cells was also diffusely stained. Pericytes of blood vessels and myoepithelial cells of salivary glands and breast were clearly defined by positively stained smooth muscle cells and pericytes; this method proved superior to conventional elastic tissue staining for studying pathological conditions of blood vessels, such as vasculitides or invasion by neoplasms. Participation of myoepithelial cells in neoplasms of the breast and salivary glands could also be evaluated. This method can provide information about histogenesis and differentiation of a variety of soft-tissue neoplasms, in conjunction with other immunohistochemical methods for various tissue-specific markers.

Actins↗
PubMed2005

Hemangioma versus vascular malformation: presence of nerve bundle is a diagnostic clue for vascular malformation.

CONTEXT: Arteriovenous vascular malformations and hemangiomas are benign vascular lesions that are difficult to distinguish from one another clinically. Also, they may be confused with each other at histopathology. Therefore, histochemical stains for the presence of an artery are frequently used to distinguish between the two. OBJECTIVE: Because it is clinically relevant to differentiate between arteriovenous vascular malformations and hemangiomas, this study was carried out to explore additional diagnostic clues that may help in the diagnosis and differentiation of these lesions. DESIGN: A total of 167 cases of benign extracranial vascular lesions were retrieved from the anatomic pathology file of our institution. These comprised 66 cases diagnosed as arteriovenous vascular malformations and 101 cases previously diagnosed as hemangiomas. The hematoxylin-eosin-stained glass slides were reviewed, Movat pentichrome histochemical stain was used to identify elastic vessels (arteries/arterioles), and S100 immunostain was used to identify nerves within these vascular lesions. For immunohistochemistry, the avidin-biotin detection method was used. RESULTS: With Movat stain, the presence of thick-walled elastic arteries was detected in 12 of the 101 cases previously diagnosed as hemangiomas, and these cases were therefore reclassified as vascular malformations. Using the same criterion, 2 of the 66 cases originally diagnosed as arteriovenous vascular malformations were reclassified as hemangiomas because they lacked arterial structures. Thus, with this strict criterion, we ended up with 91 cases of hemangiomas and 76 cases of arteriovenous vascular malformations. Intralesional nerves were identified in 91% (69/76) of cases of arteriovenous vascular malformations, including all the 12 arteriovenous vascular malformations previously diagnosed as hemangiomas. In contrast, no intralesional nerve was detected in any of the 91 hemangiomas. CONCLUSIONS: These results show that nerve bundles are consistently present in vascular malformations and absent in hemangiomas and so can be used as a diagnostic clue to differentiate between these lesions. Also, in addition to describing a previously unreported component of vascular malformations, these data further confirm the hamartomatous nature of these lesions.

Arteries↗
PubMedDate not supplied

Extrarenal malignant rhabdoid tumour of the heel--a case report.

Malignant rhabdoid tumour is a rare soft-tissue neoplasm that occurs in children and young adults. Cases have involved numerous extrarenal sites, including the thymus, liver, paravertebral region, central nervous system, heart, prostate, pelvis, chest wall, extremities, as well as soft tissues. Given their highly aggressive nature, early diagnosis of rhabdoid tumours is essential; indeed, survival times after initial presentation are typically in the range of months rather than years. The low incidence of rhabdoid tumours and confusion between this type of tumour and other tumours has precluded the collection of adequate data on its clinical presentation and there is no specific therapeutic protocol. Owing to the rarity of this condition and the difficulties encountered in its diagnosis and treatment, we report the case of a malignant tumour with rhabdoid-like features in the foot that confirms the local aggressiveness and the high metastatic potential of this type of tumour.

Adult↗
PubMed1985

Myelin basic protein and P2 protein are not immunohistochemical markers for Schwann cell neoplasms. A comparative study using antisera to S-100, P2, and myelin basic proteins.

Immunohistochemical localization of tissue specific or cell-specific antigenic markers in neoplastic cells has become an increasingly important tool in the pathologic diagnosis of tumors. The myelin-specific proteins of peripheral nervous system myelin, because they are normally synthesized in Schwann cells, are potentially useful markers for neoplasms arising from peripheral nerves. The authors carried out immunohistochemical studies on 18 cases of Schwann cell neoplasms, including schwannomas, neurofibromas, and granular cell tumors, to determine whether two myelin-specific proteins, myelin basic protein and P2 protein, were present in neoplastic Schwann cells. None of these tumors showed immunostaining for either myelin basic protein or P2 protein in neoplastic cells. In contrast, S-100 protein, which is a well established marker for normal and neoplastic Schwann cells, was localized by immunohistochemistry to neoplastic cells in all 18 neoplasms. Therefore, although myelin basic protein and P2 protein are known to be Schwann-cell-specific proteins, they do not appear to be expressed commonly in neoplastic Schwann cells.

Histocytochemistry↗