João Luiz Vitorino Araújo, MD,* Guilherme Brasileiro de Aguiar, MD,* Ulisses do Prado Aguiar, MD,* Denilson Mayrink, MD,Þ Nelson Saade, MD,* and José Carlos Esteves Veiga, MD, PhD*
Abstract: Malignant chondroid syringoma is a mixed cutaneous tumor, with epithelial and mesenchymal components, which compromises principally the trunk and extremities. This lesion is quite rare, with few cases related in the literature and no publications demonstrating its involvement of the central nervous system. Histologically, owing to its mixed origin, it represents a lesion that is difficult to recognize, often being confused with basocellular carcinoma. We report the case of a female patient, carrier of malignant chondroid syringoma in the occipital region, with invasion of the central nervous system, who was submitted to surgical excision of the lesion at our service. We also made a brief revision of the literature on the theme.
Malignant chondroid syringoma is a mixed cutaneous tumor, with epithelial and mesenchymal components,1Y3 which compromises principally the trunk and extremities.1,4 This lesion is quite rare,1Y6 with few cases related in the literature and no publications demonstrating its involvement of the central nervous system. We present the case of a female patient, carrier of malignant chondroid syringoma in the occipital region, with invasion of the central nervous system, who was submitted to surgical excision of the lesion at our service. We also made a brief revision of the literature on the theme.
Our patient, a 31-year-old woman, was admitted to our neurosurgery service presenting with a visual deficit (severe amblyopia), occipital headache, and progressive muscular strength reduction in the left superior member beginning 2 months before admission, as well as evident occipital ulcerative lesion that had been in progress for 4 months. One week before hospitalization, she had presented with bilateral visual turbidity, with rapid progression to amaurosis. On physical examination, she presented with a hardened ulcerated lesion in the occipital region, with deep plane infiltration and elimination of tenuous purulent secretion (Fig. 1A). On neurologic examination, she presented with dysmetria to the left, grade 3 left brachial monoparesis, and bilateral amaurosis associated with papilledema. Cranial computed tomography and magnetic resonance imaging were performed, and they showed expansive infiltrative occipital lesion with osseous erosion and intensive vasogenic edema in the occipital lobe (Figs. 1BYD). The patient underwent emergency surgery for the excision of the occipital expansive process, associated with the confection of a cutaneous patch and adaptation of the aponeurotic galea in the dural lesion. The histopathologic and immunohistochemical diagnoses established it as a malignant chondroid syringoma (Fig. 2). The patient was released from neurosurgery on the eighth postoperative day and was referred to the oncology service for complementary therapy. She presented with a discrete improvement in the visual condition and total recuperation from the superior member paresis.
The chondroid syringoma is a mixed cutaneous tumor with epithelial and mesenchymal components.1Y3 The term chondroid syringoma was proposed because of the invariable presence of sweat gland elements and the prominent component of cartilage-like material in most tumors. It possesses benign and malignant variables.1,4 The malignant variable, reported in the present case, constitutes a rare tumor lesion,1Y5 originating mainly in the sweat glands, its malignancy commonly arising from its epitheleal component.1 Histologically, owing to its mixed origin, it represents a lesion that is difficult to recognize, often being confused with basocellular carcinoma.1 Optical microscopy permits the observance of tubular epithelial cells that form nets or strings and keratinocyte cysts.1,3,5 Its stroma is essentially fibrous and presents myxoid chondroid alterations.2,3 Owing to its histologic pleomorphism, the differentiation between the benign and malignant variables becomes difficult.1 Malignant lesion is suspected when there are necrosis, infiltrative margins, cellular atypia, or satellite nodules.1 In the immunohistochemical evaluation, malignant lesions present positivity for glial fibrillary acidic protein, cytokeratin, and protein S-100.1,2,4 Cutaneous lesion progresses slowly, often being confused with its benign variant.2,3 Malignant chondroid syringoma more commonly occurs in women in their sixth decade of life, mostly compromising the trunk and the extremities.1,2,4,6 Different from the literature, the present case presents in a woman in her third decade of life and involves her skull, which makes this presentation even rarer. Generally, it is larger than the benign form.6 The benign variant is more common and originates in the salivary glands.1 Different from the malignant type, it more often presents in men in their fourth decade of life, frequently involving the head and neck.1 Disseminated metastasis of the malignant chondroid syringoma more frequently occurs in the lymph nodes, lungs, and bones.1Y3,
To the best of our knowledge, there is no report of central nervous system involvement by malignant chondroid syringoma, be it primary or metastatic. There are only reports of metastases present in the scalp.2 The primary treatment of this tumor, as occurred in this case, consists of radical excision of the lesion with safety margins and resection of the compromised lymph nodes.3 Complementation of the surgical treatment with radiotherapy is still controversial and does not present evidence in support of its routine use or even in relapse cases.3,4 As in the primary disease, there is no consensus on the possible use of radiotherapy or chemotherapy, which has not presented good results, in metastatic disease.3,4
Malignant chondroid syringoma constitutes a rare entity that necessitates precise histopathologic analysis for its correct diagnosis. Its early identification is important owing to the frequency of metastases and to the possibility of prolonged survival if its surgical excision is performed before the onset of secondary lesions.