Malignant Melanoma and Atypical Fibroxanthoma: An Unusual Collision Tumour

ABSTRACT Two different neoplasms in the same biopsy material, called collision tumour, were studied. These tumours are rarely seen in the skin. We report the case of a 79-year-old female with a collision tumour composed of amelanotic malignant melanoma and atypical fibroxanthoma of the face. The histological and immunopathological features observed are discussed.

Fibroma Xantoma Atípico a Propósito de un caso de una mujer en edad mediana / Atypical Xanthoma Fibroma Based on a case of a middle-aged woman

RESUMEN El fibroxantoma atípico es un raro tumor mesenquimatoso de malignidad intermedia. Se lo considera en la actualidad una variantesuperficial delhistiocitoma fibroso maligno. Afecta en su mayoría a pacientes de edades avanzadas y con historia de foto daño. El pronóstico es favorable con la extirpación completa de la lesión. Exhibeíndices bajos de metástasis. Presentamos el caso de una paciente con clínica, histología e inmunomarcación compatibles con fibroxantoma atípico.El interés de esta presentación, se fundamenta en la escasa prevalencia documentada de esta patología en la adultez, sin evidenciar participación infiltrativa del tejido óseo-medular en el control evolutivo.

SUMMARY Atypical fibroxanthoma is a rare mesenchymal tumor of intermediate malignancy. It is currently considered a superficial variant of undifferentiated pleomorphic sarcoma. It mostly affects elderly patients with a history of photo damage. The prognosis is favorable with the complete removal of the lesion. Exhibits low rates of metastasis. We present the case of a patient with symptoms, histology and immunostaining compatible with atypical fibroxanthoma. The interest of this presentation is based on the low documented prevalence of this pathology in adulthood, without evidence of infiltrative participation of bone-marrow tissue in evolutionary control.

Fibroxantoma atípico en un anciano / Atypical fibroxanthoma in an elderly

Se describe el caso clínico de un anciano de 72 años de edad, quien presentaba un tumor cutáneo dorsal maligno, por lo cual fue atendido de forma multidisciplinaria en 2 hospitales de la provincia de Santiago de Cuba. Dado el diagnóstico preoperatorio de epitelioma basocelular de piel se procedió a la extirpación del tumor de forma ambulatoria. El resultado del estudio anatomopatológico no confirmó dicho diagnóstico, pero sí corroboró que se trataba de un fibroxantoma atípico. Con los márgenes quirúrgicos de seguridad amplios se logró la curación del paciente.

The case report of a 72 years old man who presented a dorsal cutaneous neoplasm is described, reason why he was assisted in a multidisciplinary way in 2 hospitals from Santiago de Cuba. Given the preoperative diagnosis of skin basal cell epithelioma the tumor was removed in an ambulatory way. The result of the pathologic study didn't confirm this diagnosis, but it corroborated that it was an atypical fibroxanthoma. With the wide surgical margins of security the patient's cure was achieved.

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

Paediatric Primary Pachymeningeal Xanthogranuloma with Scattered Foci Displaying Reticulohistiocytoma-like Features

We report a unique case of a 4-year-old girl with an intriguing fibrohistiocytic tumour. Magnetic resonance imaging scans showed a dural mass of variegated intensity compressing the left occipital pole and apparently extending toward the superior sagittal sinus. Grossly, the cut surface of the surgical specimen was yellow, pale, and soft with reddish kernel-like crusts. Histologically, the yellow areas resembled cholesterol granulomas with widespread coagulative necrosis, cholesterol clefts, powdery calcification, foreign body-type giant cells, and foamy macrophages, while the scattered red spots contained numerous multinucleated giant cells of foreign-body and Touton types, the former with amphophilic to slightly eosinophilic cytoplasm. Immunoperoxidase reactions confirmed the expression of histiocytic markers and vimentin. As far as we know, no tumour displaying these peculiar morphological features has yet been described.

Atypical Fibroxanthoma in Head and Neck

Atypical fibroxanthoma is a pleomorphic spindle cell tumor of the dermis and it's been known to be a benign lesion clinically in spite of malignant histologic features. But recurrence is estimated at between 2%-20% and metastasis has been reported. We are about to describe a 70-year-old man with the lesion developed on the left infra-auricular area. The lesion was located superficially and is composed of compact pleomorphic spindle cells and several bizarre multinucleated giant cells. The patient was treated with wide excision. We would like to discuss about this case with a review of literatures.

Atypical Fibroxanthoma Masquerading as Xanthogranuloma / 대한피부과학회지

Atypical fibroxanthoma is an uncommon spindle cell tumor that has a clinically benign course despite the presence of bizarre xanthoma cells. A 90-year-old female presented with a 2-month history of an ulcerative plaque on the right cheek. The initial punch biopsy revealed a benign histological appearance with prominent xanthomatous cells, but the specimen excised afterwards showed malignant features with a spindle and epithelioid cell proliferation with pleomorphism and atypia. The tumor cells stained positively for vimentin and CD68, they were partially positive for CD99 and CD10 and they were negative for CD34, S100, p63, desmin and actin. We report here on a case of atypical fibroxanthoma masquerading as xanthogranuloma.

Intracranial Fibroxanthoma in an Infant: a Case Report

A primary fibroxanthoma of the central nervous system is very rare. We present a case of an infantile fibroxanthoma that arose from the cranial dura mater in a six-month-old girl with US, MRI and PET/CT features that mimicked a meningioma. The tumor appeared as a large, well-circumscribed echogenic mass in the right parieto-occipital area on US. The tumor was seen as isoattenuated to slightly hypoattenuated on pre-contrast CT scan and as hypometabolic on PET/CT. As seen on T2-weighted image, the mass was heterogeneously hyperintense to the gray matter. The mass was isointense on T1-weighted image and homogeneously strongly enhanced on contrast enhanced T1-weighted image.

A Case of Recurrent Atypical Fibroxanthoma Treated with Mohs Micrographic Surgery / 대한피부과학회지

Atypical fibroxnathoma (AFX) is a pleomorphic spindle cell neoplasm of the dermis, and is a clinically-benign reactive lesion, despite apparently malignant histologic features. We describe a case of 16-year-old female with recurrent AFX which developed on the occipital scalp. The lesion was totally excised by Mohs micrographic surgery and no recurrence was observed during one year follow up period. It is uncommon for AFX, such as the present case, to develop on a non-sun-exposed area and in adolescence.

Atypical Fibroxanthoma of Scalp Involving Occipital Bone

Atypical fibroxanthoma is a pleomorphic spindle cell neoplasm characterized by a variable combination of cells with fibroblastic and histiocytic features. It occurs mostly on sun-exposed area of the head and neck of elderly person and is a clinically benign reactive lesion despite apparent malignant histologic features. However, because of its potential for metastasis, it is widely regarded as a low-grade sarcoma. We report a 30-year-old woman with atypical fibroxanthoma developed on the left occipital area. The lesion was 1.5 x 2 cm sized papule. There was no skin lesion such as ulcer or eschar. However, mass was involving occipital bone and composed of dense, pleomorphic spindle cells and several bizarre multinucleated giant cells. After wide excision of the scalp and occipital bone, the defect was covered with bone cement, bipedicled local flap and the donor site was covered with STSG. The wound healed completely without complication. It remained free of recurrence for a period of about 1 year follow up.

A Case of Atypical Fibroxanthoma

Atypical fibroxanthoma(AFX) is a tumor that occurs as a solitary and ulcerative nodule on actinically damaged or radiation damaged skin of the head and neck in the elderly. AFX is a pleomorphic spindle cell neoplasm of the dermis, which is a clinically benign reactive lesion despite of apparently malignant histologic features. We report a case of AFX which developed on the nasal root area of a 23-year-old man. This case is very unusual in terms of site(less sun-damaged area), overlying skin feature(grossly normal skin) and age(too young). This tumor was completely removed with surgical excision and remained free of recurrence for a period of about 1 year follow-up.

A Case of Atypical Fibroxanthoma / 대한피부과학회지

Atypical fibroxanthoma(AFX) is a pleomorphic spindle cell neoplasm of the dermis, which is a clinically benign reactive lesion despite apparently malignant histologic features. We describe a 38-year-old woman with AFX developed on the right popliteal area. The lesion was superficially located and composed of densely cellular, pleomorphic spindle cells and several bizarre multinucleated giant cells. It is uncommon that AFX developes on the lower leg in the middle age.

A case of multiple bowen's disease associated with adenoid basal cell carcinoma / 대한피부과학회지

AFX is a fibrohistiocytic tumor which usually shows benign clinical rocess despite malignant histolgy. We report a case of AFX showing storiform pattern distribution of tumor cells which developed on the right temple area of a 70-year-old woman.

Two cases of atypical fibroxanthoma of the skin / 대한피부과학회지

Atypical fibroxanthoma(AFX) occurs most on sun-exposed area of the head and neck of elderly person. It has an excellent prognosis after conservative, but complete, excision. However, because of its potential, albeit small, for metastasis, it is widely regarded as a low-grade sarcoma. We present herein two cases of atypical fibroxanthoma. The case 1 was a 86-year-old female who had a small egg-sized, dome shaped nodule with eroive surface on the left cheek. The other case was a 60-year-old male who had solitry bean-sized, nodulo-ulcerative lesion on the vertex. Immunohistochemical studies revealed positive reaction for vimntin and a-antichymo trypsin. These patients have received completely total surgical ecis on and remained free of recurrence for a period of about, 2 years follow up.

Atypical Fibroxanthoma of the Skin / 대한피부과학회지

An immunohistochemical and electron microscopic study of an atypical fibroxanthoma of the skin (AFX) was reported. The lesion developed on the left scapulararea in a 70-year-old man. The result using antibody to S-100 protein was negative and the tumor cells observed with electron microscope were undifferentiated, fibroblast-like, histiocyte-like and multinucleated giant cells. But Langerhans-like cells or Langerhans were not found.