Intranodal palisaded myofibroblastoma: a case report
© Karvouni et al; licensee BioMed Central Ltd. 2010
Received: 5 November 2009
Accepted: 2 February 2010
Published: 2 February 2010
Intranodal palisaded myofibroblastoma is a rare benign soft tissue tumor, almost always arising from inguinal lymph nodes. It usually presents as a painless, slow-growing inguinal mass. We report herein a case of an intranodal palisaded myofibroblastoma occurring in a 36-year-old man. The salient clinicopathologic features of this unusual tumor are presented and the literature is briefly reviewed.
Intranodal palisaded myofibroblastoma (IPM) also called as intranodal hemorrhagic spindle cell tumor with amianthoid fiber is a rare benign tumour of the lymph node that may be derived from myofibroblasts or smooth muscle cells. The most usual area of presentation is the inguinal lymph nodes, but occurrence within other areas such as mediastinum and submandibular lymph nodes has also been reported . This unusual lesion was first well characterized in 1989. Previous reports of similar cases in the literature were considered to represent intranodal primary or metastatic neurilemoma. To our knowledge, approximately 53 such cases have been reported in the literature . The ages of patients ranged from 19 to 78 years with a slight male predominance [3, 4].
Intranodal palisaded myofibroblastoma is a benign tumor which has a great predilection for the inguinal lymph nodes. Submandibular and cervical lymph nodes have also been reported as rare originating sites . It was first well characterized in 1989 in two series of patients that were reported in the literature under the names of palisaded myofibroblastoma  and intranodal hemorrhagic spindle cell tumor with amianthoid fibers . Clinically, it presents as an asymptomatic, slowly growing tumor. It is more common in males and it is diagnosed at a wide age range (from 19 to 78 years) . Its size varies from 0.6 to 5 cm .
Its predominant morphologic features include the bland appearance of its constituent spindle cell population and the presence of acellular eosinophilic stellate areas (so-called amianthoid fibers). As an intranodal lesion, intranodal palisaded myofibroblastoma grows from the interior portion of the lymph node expanding outwardly to the periphery; thus, normal lymphoid tissue is compressed to the periphery.
The immunohistochemical profile of the neoplastic cells along with their ultrastructural features is indicative of myofibroblastic or smooth muscle differentiation [5, 6]. Intranodal palisaded myofibroblastoma has recently been shown to have a strong expression for cyclin D1 and a low proliferating index of Ki- 67 . The overexpression of cyclin D1 presented in the study of Kleist et al, points to the proliferation regulatory pathway as one of the factors involved in the etiologic pathogenesis of intranodal palisaded myofibroblastoma . It is negative for neural markers such as S100, and endothelial markers such as CD34, and desmin.
The presence of a spindle cell neoplasm in a lymph node requires a thorough clinical work - up to identify a primary neoplasm. Metastatic spindle squamous cell carcinoma, melanoma, sarcomatoid renal cell carcinoma are examples of carcinomas that can present as spindle cell lymph node lesions. Metastatic sarcomas should also be included in the differential diagnosis. Spindle cell tumors that arise primarily in lymph nodes are Kaposi sarcoma, dendritic cell tumor and benign mesenchymal tumors such as neurofibroma . Schwannomas are unlikely to occur within the lymph nodes, due to the fact that they arise from Schwann cells of the nerve sheath, which do not innervate lymph nodes. Furthermore, intranodal palisaded myofibroblastoma lacks Antoni A type A and B areas and does not express S100 protein. Nuclear palisading, amianthoid-like changes and the lack of a history of immunocompromised status distinguish intanodal palisaded myofibroblastoma from Kaposi sarcoma. In order to exlude hemangioendothelioma from the differential diagnosis, expression of endothelial markers such as CD34 and CD31 should be performed during the immunohistochemical analysis. Finally, one must rule out the possibility of metastases from malignant melanoma and sarcoma based on thorough clinical history and an appropriate immunohistochemical profile.
Although a wide range of soft tissue tumors can be included in the differential diagnosis, intranodal palisaded myofibroblastoma has a distinctive morphological feature and an immunohistochemical profile. When the diagnosis is suspected, it is best to report this lesion as a low grade spindle cell tumor and recommend surgical excision. Excellent prognosis has been reported after surgical treatment with a 6% recurrence rate and no malignant transformation .
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the journal's Editor-in-Chief.
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