[Cellular fibrous histiocytoma: a clinicopathologic analysis of 27 cases].

OBJECTIVE: To study the clinicopathologic characteristics of cellular fibrous histiocytoma (CFH) with emphasis on diagnosis and differential diagnosis. METHODS: Clinical and pathologic features were reviewed in 27 cases of CFH (encountered during the period from 2008 to 2012) along with outcome analysis. Immunophenotyping was performed with EnVision method. RESULTS: The patients included 13 males and 14 females. The age at presentation ranged from 15 to 61 years (mean, 34 years; median, 32 years). The tumor occurred in the extremities (n = 14), head and neck (n = 7), and trunk (n = 6). Histologically, the tumors were located in the dermis. Some cases showed wedge like extension into the subcutaneous adipose tissue. On high power, they consisted of dense fibroblasts and myofibroblasts. Other cell components such as psammoma-like histiocytes, hemosiderin-containing macrophages or touton-type giant cells were rare. The spindled tumor cells were arranged mostly in intersecting fascicles. Focal storiform architecture was not uncommon. In addition, a few cases showed prominent hemangiopericytoma-like pattern. There was no prominent cellular atypia but increased mitotic figures were not difficult to find. Two cases exhibited necrosis. By immunohistochemistry, the tumor cells showed variable expression of alpha smooth muscle actin. CD34 positive cells were present in some cases, but were distributed mostly in the periphery or bottom of the lesions. They were all negative for desmin, h-caldesmon, S-100 protein and cytokeratin. Follow-up in 19 cases revealed local recurrences in 5 cases and bilateral pulmonary metastases in 1 case after repeated recurrences. CONCLUSIONS: CFH is a cellular form of benign fibrous histiocytoma which has a risk for local recurrence after incomplete excision. Distant metastasis can occur in rare examples. However, there were no morphological parameters predicting the risk of recurrence or metastasis. Increased awareness of the clinocopathological features and immunophenotypes of CFH is helpful in avoiding misdiagnosing the disease as malignant tumors, especially dermatofibrosarcoma protuberans.

Metastasizing "benign" cutaneous fibrous histiocytoma: a clinicopathologic analysis of 16 cases.

Cutaneous fibrous histiocytoma (FH) is considered a benign tumor; however, certain types of FH have been shown to have a tendency for local recurrence, and there are rare reported cases of metastasis. In this study, 16 cases of morphologically benign FH with locoregional or distant metastasis were identified in consult files. Pathologic features of primary, recurrent, and metastatic tumors, as well as clinical outcome, were evaluated. Nine were male and 7 were female patients; mean age was 42 years (range, 3 to 68 y). Primary tumors arose on the leg in 5 patients, buttock in 1, trunk in 3, shoulder in 3, neck in 2, and finger in 1. The primary site in 1 case was unknown. Fifteen primary tumors available for review involved the dermis; 6 extended into the superficial subcutis. Tumor size ranged from 1 to 5 cm (median 3.2 cm). Histologically, primary tumors showed characteristic features of FH, being composed in most cases of a polymorphous population of bland spindle and histiocytoid cells in a mixed storiform and fascicular growth pattern with admixed foam cells, multinucleate cells, and inflammatory cells in varying proportions. Histologic variants included 11 cellular (2 with mixed atypical and cellular features), 2 aneurysmal, 1 atypical, and 1 epithelioid type. All tumors showed entrapment of hyalinized collagen bundles. Mitotic activity ranged from <1 to 13/10 HPF. Focal necrosis was seen in 1 primary tumor. Ten patients had local tumor recurrence; 4 patients had multiple local recurrences. Time to first recurrence ranged from 6 weeks to 13 years. The local recurrences of 1 tumor showed increased cytologic atypia, but recurrences were otherwise morphologically similar to primary tumors. Metastases occurred 0 to 180 months after diagnosis (median 17 mo) and involved the lungs (12 patients), lymph nodes (8), soft tissues (6), and liver (1). Five patients developed multiple satellite nodules in the region of the primary tumor. Metastases were morphologically similar to the primary tumors. So far, 6 patients died of disease, with a median time to death of 64 months (range, 10 to 168 mo). Four patients are alive with metastatic disease. Two patients are disease free at last follow-up, and 1 patient died of unrelated disease. Metastasis of morphologically benign cutaneous FH is an extremely rare but clinically aggressive event. Primary tumors tend to be large and cellular, but aggressive behavior cannot be predicted on morphologic grounds alone; however, early or frequent local recurrence may warrant closer clinical follow-up.

Clinical spectrum of atypical fibroxanthoma and undifferentiated pleomorphic sarcoma in solid organ transplant recipients: a collective experience.

BACKGROUND: Atypical fibroxanthoma (AFX) and undifferentiated pleomorphic sarcoma (UPS) are uncommon, spindle cell cutaneous malignancies. Solid organ transplant recipients (SOTRs) are immunosuppressed and therefore have a higher incidence of cutaneous malignancies. OBJECTIVE: We describe the clinical spectrum of AFX and a more-aggressive, deeper variant, UPS, in SOTRs. MATERIALS AND METHODS: A retrospective chart review of AFX and UPS in SOTRs was implemented. Cases from Vanderbilt University, Emory University, Mayo Clinic-Jacksonville, and University of Rochester were included. A literature search included previously published cases. RESULTS: The average age of SOTRs at time of tumor presentation was younger than typically seen in immunocompetent patients for AFX. Rates of local recurrences and metastases were higher in the SOTRs than is noted in the immunocompetent literature. Rates of recurrence were higher in those treated with excision than in those treated with Mohs micrographic surgery (MMS). CONCLUSION: AFX and UPS may have a greater risk for recurrence, metastases, and mortality in SOTRs, in whom early treatment with MMS may demonstrate certain advantages in terms of minimizing risk of recurrence and metastasis. UPS and recurrent tumors should be staged appropriately and may respond to adjuvant radiation therapy and reduction of immunosuppression. Immunohistochemical evaluation is recommended to exclude other spindle cell tumors.

Benign fibrous histiocytoma of the skin metastasizing to the inguinal lymph node.

Benign fibrous histiocytoma (FH, dermatofibroma) is a common skin lesion but its metastasizing variant is extremely rare and only a few cases have been reported to date. The usual sites of metastases include locoregional lymph nodes and lung. In the majority of cases, the clinical course is indolent. At present, there are no reliable clinical or histological features of the primary tumour that could predict the risk of locoregional or distant metastases. Authors describe a case of metastasizing FH and briefly review available data.

Atypical fibroxanthoma with regional lymph node metastasis: report of a case and review of the literature.

BACKGROUND: Atypical fibroxanthoma (AFX) is a low-grade sarcoma usually occurring on sun-damaged skin of the head and neck in elderly patients. Metastatic disease has been reported very rarely. The potential aggressiveness of AFX is controversial. OBSERVATIONS: We describe herein a patient who developed metastatic disease in cervical lymph nodes. Our patient was an 87-year-old man with a 7-week history of a rapidly growing AFX presenting as a 1.5-cm sessile nodule on his right mandible. Two months following excision, the patient developed cervical lymphadenopathy. Histopathologic analysis of the cervical lymph nodes revealed spindle-cell tumors with histologic characteristics identical to those of the primary AFX, and the tumors were immunonegative for cytokeratin MNF-116 and S-100. In addition, we review and analyze cases from the literature and articles related to immunohistochemical stains used to diagnose AFX. CONCLUSIONS: Atypical fibroxanthoma is a diagnosis of exclusion, and only a small number of metastatic AFX cases have been reported. A review of the literature pertaining to immunohistochemical stains suggests the potential benefit of use of CD10, procollagen I, CD99, CD117, p63, and LN-2 in differentiating AFX from other spindle-cell tumors. The metastatic potential of AFX may not be fully appreciated, and clinicians should be reminded of its potential aggressive behavior.

Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a rare soft-tissue tumour of uncertain differentiation and low metastatic potential. Cytogenetics and/or molecular genetics have revealed that most have a rearrangement of the EWSR1 gene, whereas a FUS gene rearrangement is present in a minority of cases. Although some cases of AFH display striking pleomorphism and mitotic activity, there are no known clinical, morphological or genetic factors that predict metastasis. The authors present clinicopathological features of AFH, including cases showing a pleomorphic histological appearance, and results of fluorescence in situ hybridisation analysis of EWSR1 and FUS rearrangements. METHODS: Tumour samples from 10 patients were subjected to clinicopathological and immunohistochemical analysis and dual-colour fluorescence in situ hybridisation for EWSR1 and FUS with split-signal probes. RESULTS: All cases showed clinical features (sites: extremities followed by trunk; age: adolescent to young adult), morphology (multinodular proliferation of spindle cells, lymphoid cuffs and pseudovascular spaces) and immunohistochemical results (more than half were positive for CD68, CD99, desmin and epithelial membrane antigen) typical of AFH. There were two local recurrences in each of two patients. Two patients developed distant metastases and died from the disease; tumours of these two patients showed focal proliferation of large pleomorphic cells with hyperchromatic nuclei and high proliferative activity (>10/10 high-power field and Ki-67 labelling index >10%). There were no clinical, histological or immunohistochemical differences between the nine cases with EWSR1 rearrangement and one case with FUS rearrangement. CONCLUSIONS: Wide surgical excision and careful follow-up are necessary for patients with AFH in view of its risk of local recurrence and metastasis leading to a fatal outcome.

Sclerosing haemangioma with lymph node metastasis.

Sclerosing haemangioma (SH) of the lung is an intrapulmonary tumour composed of polygonal and cuboidal cells arranged in a heterogeneous pattern. SH is generally regarded as benign or very low-grade; only rarely have cases of lymph node metastasis been reported. We report a case of SH with metastasis to the regional lymph nodes in an 18-year-old male, whose left lung tumour was identified incidentally on routine CXR. The left upper lobe was removed and the regional lymph nodes dissected. The tumour consisted of proliferative cuboidal and polygonal cells arranged in a papillary pattern within the sclerotic stroma. Subcapsular metastasis was observed in a lymph node. SH with lymph node metastasis was diagnosed. The patient was disease-free at follow up after 2 years. Lobectomy with nodal dissection is suggested for the treatment of pulmonary SH, especially in the case of large tumours with enlargement of regional lymph nodes. Investigation of more cases and long-term follow up are necessary to assess the clinical significance of lymph node metastasis.

[Dermatofibroma metastasizing to the lung: current treatment]. / Dermatofibroma y metástasis pulmonares. Tratamiento actual.

Dermatofibromas are very common skin tumors. Their typical presentation is as a slow-growing, firm, solitary papule. They have been described histopathologically as a reaction of the connective tissue of the skin or as a benign neoplasm. Cases of these tumors metastasizing to the lung were first reported in 1990. Since then 12 cases with similar characteristics have been reported worldwide. We present the case of a young woman with a recurrent dermatofibroma on the shoulder that metastasized to both lungs. We discuss the histologic characteristics of this tumor that could raise suspicion of unusually aggressive behavior.

[Difficulty in diagnostic procedure in the malignant fibrous histiocytomas of larynx]. / Trudnosci diagnostyczne w przebiegu miesaka polimorficznego krtani.

Malignant fibrous histiocytomas (MFH) of the upper respiratory tract are rare, aggressive mesenchymal neoplasms. Ultrastructurally MFH consisted of five different types of cells. It occurs principally as a mass of the extremities, abdominal cavity, or retroperitoneum in adults. There have been sporadic laryngeal cases reported in the literature. We report a case of a glottic malignant fibrous histiocytoma on a 45 year old man. There was difficulty to find finally diagnosis. Pathologic and immunohistochemical evaluation revealed tumor a few months after first symptoms. There was unilateral metastases into the neck lymph nodes. Immunohistochemical staining was positive for F13a, CD68 and negative for desmin and cytokeratin. Wide, aggressive excision of the tumour with total larygectomy to be the treatment of choice because it was difficult to prove clinically and pathologically that it was MFH. Unilateral neck dissection was done due to metastases into the neck lymph nodes. Radiation have been used after surgery. About two years after total laryngectomy the patient is well and free of disease.

Metastasizing dermatofibroma in lung.

Benign dermatofibroma is very common skin tumor and can very rarely metastasize. We report a case of metastasizing dermatofibroma on a 36-year-old woman who presented multiple bilateral lung nodules. She underwent incisional biopsy for cellular dermatofibroma of the right shoulder 7 years ago. Chest computed tomographic scanning shows multiple nodules in both lung fields. Segmental and wedge resections were done. Grossly, the masses were hemorrhagic cysts. Microscopically, there were dilated cystic airspaces. The airspaces were lined by respiratory and metaplastic squamous epithelium with underlying layers of fibrohistiocytic spindle cells with storiform and fascicular pattern. The tumor cells stained for CD68 and CD10. The lung mass shows same histologic features with skin lesion.

Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases.

BACKGROUND: Fibrosarcomatous (FS) or malignant fibrous histiocytomatous (MFH) transformation of dermatofibrosarcoma protuberans (DFSP) is a rare, but well known, entity. DFSPs with sarcomatous areas have questionable biological behaviour. Several studies suggest that they have a higher risk for local recurrence and distant metastases than ordinary DFSPs. One recent study described no difference in the behaviour of conventional and transformed DFSP. AIMS: To investigate the biological behaviour of a series of transformed DFSPs. METHODS: Eight transformed DFSPs were analysed clinicopathologically. Follow up ranged from four to 36 years. RESULTS: The tumours involved the trunk (six cases) and lower extremity (two cases) and measured 3.5-8 cm (median, 4). Sarcomatous change presented de novo in all cases. The type of sarcomatous change was FS (five cases) and MFH (three cases). The estimated proportion of sarcomatous area in the tumour was 25-70% (median, 43.37%). Mitotic counts ranged from nine to 16 mitotic figures/10 high power fields in the FS and MFH areas (median, 12), and from one to three in the DFSP areas. Six patients were treated by wide local excision with histopathologically negative margins and two were treated by simple surgical excision with positive margins. Three patients developed recurrences and one developed metastasis during follow up. Of those treated by wide local excision, one developed recurrence. All tumours expressed CD34 in the DFSP component, but only three in the sarcomatous area. CONCLUSIONS: Although DFSP containing sarcoma may be a more aggressive tumour, its behaviour can be influenced by surgical treatment.

Primary malignant fibrous histiocytoma of the jejunum metastatic to the spine: report of a case.

We report a case of malignant fibrous histiocytoma (MFH) metastatic to the spine. A 41-yr-old male was admitted to our hospital for radiation treatment of MFH of the spine. He began to show signs suggestive of partial small bowel obstruction. Computed tomography demonstrated jejuno-jejunal intussusception. The patient was taken to the operating room, where the diagnosis was confirmed. Partial jejunal resection was performed. The lead point of the intussusception was histologically diagnosed to be a high-grade malignant fibrous histiocytoma. We believe that the spinal lesion was the metastatic lesion and that metastasis occurred via the vessels of Adamciewicz. To our knowledge this is the first case thus reported.

Metastasizing atypical fibroxanthoma (cutaneous malignant histiocytoma): report of five cases.

BACKGROUND: Atypical fibroxanthoma (AFX) is an unusual malignant fibrohistiocytic tumor of sun-damaged skin. When first described, it was felt to be a reactive tumor of low malignant potential. More recently, it has been shown to be a tumor of intermediate malignant potential. OBJECTIVE: To describe five cases of metastatic AFX. RESULTS: Five patients ranging in age from 65 to 85 years old presented with metastatic AFX. Three of the five cases presented with regional lymph node disease. Also, three of the five cases had other aggressive cutaneous malignancies. LN-2 (CD74) staining was positive in three of five primary tumors and two of five metastatic tumors. CONCLUSION: The metastatic potential of AFX may be underestimated. LN-2 staining may be a useful marker in identifying more aggressive tumor behavior.

Malignant fibrous histiocytoma of the heart complicated by factor XI deficiency in a Jehovah's Witness patient.

We describe a 16-year-old girl with malignant fibrous histiocytoma (MFH) of the heart complicated by factor XI deficiency. The preoperative diagnosis was left atrial myxoma. We decided to perform the operation owing to a normal bleeding time. Operative findings suggested a malignant tumor. The patient was a Jehovah's Witness, and extensive excision was not performed because blood transfusion was not allowed. We resected as much of the tumor and left atrial appendage as possible. The pathologic diagnosis was MFH. Excessive bleeding was not observed during the operation. Bleeding time helps to determine whether a surgical procedure is indicated in patients with factor XI deficiency.

Localized cutaneous metastases from an atypical fibroxanthoma.

BACKGROUND: Atypical fibroxanthoma is a rare cutaneous spindle cell neoplasm typically found on the sun-exposed areas of the head or neck of the elderly. OBJECTIVE: We report a case of localized cutaneous metastases attributed to an atypical fibroxanthoma. METHODS: A case report and a review of the literature of metastasizing atypical fibroxanthoma are given. RESULTS: Mohs micrographic surgery was used to treat all skin lesions and currently the patient has no suspicious lesions. CONCLUSION: Metastases attributed to atypical fibroxanthoma are rare, and many prior reports may have actually been malignant fibrous histiocytoma, spindle cell melanoma, or spindle cell squamous carcinoma. Tumor depth, vascular invasion, and cutaneous tumor recurrence appear to increase risk for metastasis.