Intracranial Pleomorphic Malignant Fibrous Histiocytoma, Associated to Systemic Lupus Erythematous: A Case Report

Background: Pleomorphic Malignant Fibrous histiocytoma (MFH) is a rare neoplasms of the soft tissue and bone composed of fibroblastic and histiocytic components with mitosis figures, nuclear pleomorphism and anaplasia. Case Presentation: We presented a rare case of MFH in 44 year old woman with history of systemic erythematosus lupus and seizures. Treated with prednisone. The cerebral TAC showed a temporal mass. Craniotomy was performed and the examination of the biopsy sample revealed a giant, pleomorphic and atypical cells. Immunohistochemical analysis showed positivity for vimentin, CD68, Fascin, lysosyme and MIB-1 labeling index of 20%. Pleomorphic malignant fibrous histiocytoma was diagnosed, with involvement of the overlying temporal lobe. We report a case of MFH of the left temporal in adult. Discussion: The neuroimaging and intra-operative opinion were of a meningioma. Histopathology, Case Study Tena-Suck et al.; BJMMR, 21(9): 1-9, 2017; Article no.BJMMR.32743 2 in conjunction with immunohistochemical evaluation and proliferation marker, confirmed the diagnosis. This is the firth case reported of PMFH associated to systemic lupus erythematous and different histological patterns.


INTRODUCTION
Primary intracranial fibrous histiocytomas (FHTs) are rare entities some few cases have been reported.
The mesenchymal origin of fibrohistiocytic tumors (FHT) remains controversial [1,2]. These tumors can be benign or malignant forms. Malignant fibrous histiocytoma (MFH), is a type of sarcoma, of uncertain origin that arises both in soft tissue and bone. It has been suggested that these may originate from the perivascular pial sheath or mesenchymal cells or primitive mesenchymal cells, and blood vessel walls [2][3][4]. Kauffman and Stout were first which introduced the term of MFH in 1961. Furthermore, Fletcher published in 1992 a retrospective series of 159 cases of pleomorphic sarcoma [1]. These tumors were reassessed morphologically [1]. Histologically MFH is characterizes by giant and pleomorphic tumor cells with eosinophilic and foamy cytoplasm with nuclear atypia and mitosis features [1,2]. Histologically have been described four subtypes; Storiform-pleomorphic, Myxoid, Giant cell and Inflammatory [1]. MFH are rare intracranial tumors, they usually presented as a meningeal mass, intraaxially [5][6][7][8], mesenchymal [9], or and intraventricular location [10]. Some cases have been associated to radiotherapy [11][12][13][14][15][16][17]. Radiation-associated status and incomplete resection has been identified as independent predictors of local recurrence. One complication of local radiotherapy can development of radiation-associated sarcoma (RAS). It can originate in the CNS or arise as a metastasis from a primary extra cranial tumor [1,2]. However, the term of MFH It is very controversial and it was proposed name to undifferentiated pleomorphic sarcoma (UPS). Shirasuna K, et al. suggested that this tumor originates of neoplastic histiocytes with the high capacity to origin various types of cells, which suggested a possible stem cell origin [5]. This theory has been demonstrated by study of the gene-expression patterns of soft tissue sarcomas failed to find a clear distinction between the genes of MFH, liposarcoma and leiomyosarcoma [1].
Hein we reported a rare case of pleomorphic malignant fibrous histiocytoma or UPS in 44 years-old-woman with was treated by 12 year of systemic lupus erythematosus (SLE) and was emergency operated with a temporal mass suggestive of a meningioma.

CLINICAL CASE
A 44-year-old woman had been treated with steroids for SLE for 12 years. One year ago she presented seizures. She presented headache, nauseas, vomiting loss progressive loss of strength in right hemisphere, giddiness, and unsteadiness of gait, of six months' duration, stool or urine incontinence, or sensorineural deficit. The patient presented with a sudden loss of consciousness and vomiting and was admitted in our institution.
Neurological examination revealed normal higher mental functions, without any associated sensory deficit or cerebellar signs. However, her gait was ataxic. Hematological and biochemical parameters were within normal limits. Cerebral TAC revealed a well-defined, intensely enhancing extra-axial lesion along the inferior aspect of the left temporal lobe suggestive of a meningioma (Fig.  1). There was intense homogeneous postcontrast enhancement, with focal non-enhancing intralesional areas. No dural attachment was seen. She underwent left temporal craniotomy and Simpson Grade 2 excision of the tumor. Intra-operatively, the neoplasm was highly vascular, soft and necrotic areas shown.
Microscopic examination of the resected specimen showed various cell patterns; isolated classic giant cells with pleomorphism and cellular atypia, monstrous and giant cells ( Fig. 2a) with abundant eosinophilic cytoplasm (Fig. 2b), with round to elongated nuclei, few of which showed prominent nucleoli and multinuclear features (Fig. 2c), numerous atypical mitotic features were observed (Fig. 2d), with sarcomatous pattern, other pattern was formed by round cells with nucleus to the periphery rejected that looks gemistocytes vs rhabdoid cells (Fig. 2e), and the other one formed by large cells with abundant eosinophilic cytoplasm with astrocytes vs hepatoid appearance (Fig. 2f), and necrosis was also observed. Few bits showed invasion into the bony trabeculae with destruction of the bone. There were xanthomatous areas in the tumor. Follow-up. The patient has been on a regular follow-up. At the last review, she was ambulant, and with normal speech with loss of strength in right hemisphere deficit.

Fig. 3. The immunophenotyping studies. (a) Observed that the cells like hepatocytes where intensely positive vimentin showed a strong positive reaction for vimentin, (b) sarcomatous pattern the vimentin was observed positive immunoreaction only in the blood vessels, and was also positive in the rhabdoid like patterning(c). (d) Tumor was also positive staining for CD68, (e) fascin and (f) lysozyme immunoexpression were observed (IHQ stain x400)
However, the malignant form is characterizes by giant, bizarre, atypical, pleomorphic tumor cells with abundant and foamy cytoplasm, numerous mitotic figures, with a collagenous stroma [1], storiform growth pattern multinucleated giant cells may be seen. Pleomorphic MFH (PMFH) subtype is composed of fibroblasts, myofibroblasts and histiocytic-like cells [1], may represent end stage of various sarcomas with common morphologic pleomorphism features classify as MFH-giant cell [1,2]. PMFH usually shows highly aggressive behavior, recurrence, resistance to radiotherapy or chemotherapy treatment and metastasis [1]. MFH usually affect older adults (age over 50+ years) with slight male predominance; is more common location in lower extremities, rarely retroperitoneum, head and neck, breast, and usually have large and deep-seated with progressive enlargement. Some few intracerebral cases have been published [1].
By ultrastructure description relatively undifferentiated fibroblastic, myofibroblastic or primitive mesenchymal cells, some with phagocytic properties have been observed [1,3].
PMFH may have different histological patterns that complicate the diagnosis, may have features suggestive of other sarcoma types (myxofibrosarcoma, leiomyosarcoma, dedifferentiated liposarcoma, fibrosarcoma, malignant peripheral nerve sheath tumor) but it is insufficient for definitive classification [1]. Cytogenetic analyses have been revealed highly complex karyotypes lacking specific structural or numerical aberrations [18]. As well as the recurrent tumors are reported cytogenetic abnormalities, associated in chromosome bands 1p36, 1q11, 1q21, 3p12, 11p11, 17p11, and 19p13 [18], furthermore, some genes analyzed (FU-MFH-2) genes, WNT1, WISP2, G proteincoupled receptor 64 (GPR64) and Tenascin XB (TNXB) have been identified for various biologic and molecular pathogenetic behavior [18], novel approaches targeting c-Met, MEK/extracellularregulated kinase (ERK) and/or AKT should be considered for a subset of undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (UPS/MFH) [19]. Histologic and genetic advances regarding with the diagnosis of MFH being replaced by undifferentiated pleomorphic sarcoma [19]. Recently, the 2016 CNS WHO has expanded these categories to parallel those in the corresponding Hematopoietic/Lymphoid WHO classifications and are within the category of mesenchymal tumor non-meningeal, molecular parameters are used to establish brain tumor diagnoses [2]. Nevertheless, some tumors with a histological appearance more similar to traditional solitary fibrous tumor can also display malignant features and be assigned a WHO grade III [2], consequently, be required to fine-tune this grading system [2].
There is increasing evidence in support of an association between systemic lupus erythematosus (SLE) and malignancy. Lymphoma is the most common tumor associated to SLE, few reports of have been published [20], this is the firth case of PMFH associated SLE, in a patient who has not been radiated but if you have used steroids for 12 years. It is cause or effect or pure coincidence? Imaginable pathogenic paths linking SLE and cancer include supposed links with medicaments used, patients treated with immunosuppressive or steroid agents, or prolactin and viral contacts, or post-transplantation suggests and toxicity of gamma knife radiosurgery may play a role in the pathogenesis of SLE in some patients. However, risk factors like immunosuppressant, steroid and hormones could be predisposing factors for the development of meningioma during adult life are well known [21].

CONCLUSION
In conclusion, this case report documents a rare entity in an intracranial location. In a patient with SLE, with round cells with nucleus to the periphery rejected that looks gemistocytes vs rhabdoid like cells and other cells with astrocyte vs like hepacytes appearance, owing to the rarity of PMFH, one should be aware of its defining morphological and immunohistochemical characteristics for definitive diagnosis. Also genetic marker could help with the diagnosis.

CONSENT
All authors declare that 'written informed consent was obtained from the patient (or other approved parties) for publication of this paper and accompanying images.
All authors hereby declare that all experiments have been examined and approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

ETHICAL APPROVAL
It is not applicable.