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Case Presentation - Fall, 2010

Malignant Giant Cell Fibrous Histiocytoma

Patient Age and History: 83 y/o male

Specimen type: Neck Lymph Node FNA

Cytologic diagnosis: Malignant

Biopsy diagnosis: Biopsy of Left Lower Lung Lymph Node-Malignant Giant Cell Fibrous Histiocytoma.

Case provided by: The laboratory that provided this case would prefer to remain anonymous.

Malignant Giant Cell Fibrous Histiocytoma:
Malignant Giant Cell Fibrous Histiocytomas are part of a group of soft tissue sarcomas called Fibrous Histiocytomas. The lesions in this group range from benign to malignant. Benign Fibrous Histiocytoma is the least aggressive of the lesions in this group. They are common and occur in the dermis and superficial subcutaneous tissue in the extremities. FNA's are not frequently done due to the depth of the lesions. Dermatofibrosarcoma Protuberans is an intermediate grade sarcoma in this group. It occurs on the trunk and extremities. The most aggressive of the group is Malignant Fibrous Histiocytoma and is a highly malignant, high grade sarcoma. The four major sub-types are; myxoid, giant cell, inflammatory, storiform-pleomorphic (the last being the most common). Because recent investigation has shown no true histiocytic differentiation, in 2002 WHO changed the classifications. The current WHO classifications are as follows; MHF-myxoid is now classified as Myxofibrosarcoma, MFH-giant cell is now classified as Undifferentiated Pleomorphic Sarcoma with Giant Cells, MFH-inflammatory is now classified as Undifferentiated Pleomorphic Sarcoma with prominent inflammation and MFH-storiform/pleomorphic is classified as Undifferentiated High Grade Pleomorphic Sarcoma. MFH can be found in the retroperitoneum (6-16%) and more commonly the lower extremities (65-75%) mostly thigh. The most common location of metastasis is to the lung (90%). It occurs in adults between the sixth and seventh decade of life. It is the most common high grade sarcoma in adulthood. The ratio of male to female is 2:1 and occurs most frequently in Caucasians.

Cytology:
A Fine Needle Aspiration from MFH produces a very cellular specimen. There are two diagnostic cell types found; the most abundant type is described as large, spindle shaped cells occurring both singly and in loose cohesive groups. The nuclei of these cells are hyperchromatic with prominent nucleoli and are elongated or oval in shape with the cytoplasm sometimes appearing bipolar. Also present are atypical anaplastic giant cells with multiple nuclei and abundant cytoplasm, which can appear foamy. There can be necrosis and mitotic figures are a frequent finding.

Differential Diagnosis:
A diagnosis of a malignant high grade sarcoma on FNA can be easily made; however, to make a more specific diagnosis and to rule out other lesions, the use of immunocytochemistry is necessary. The histologic and cytologic pattern of MFH can be the same as other high grade sarcomas, melanoma and anaplastic carcinomas. A diagnosis of exclusion can be made favoring MFH using cytokeratin, S-100, muscle specific markers and HMB45 as well as others.

Clinical Features, Treatment and Prognosis:
The symptoms of this tumor when in the retroperitoneum are not different from other neoplasms occurring in that area and can include flank pain, gross hematuria, indigestion, nausea, abdominal distention and hernia. By the time symptoms arise, the tumor is usually quite advanced. When found in the extremities, this tumor presents as an enlarging soft tissue mass which is void of pain and usually is present less than 6 months. Surgical excision, which may include amputation, is the suggested treatment for this aggressive tumor. When located in the retroperitoneum, early and complete resection is also the appropriate treatment. Without this there will be poor prognosis. Radiation and chemotherapy may be used in some cases, but is questionable. Even with treatment the prognosis is poor when found in the retroperitoneum with a recurrence rate of greater than 50% and a 5 year survival rate of only 14%. While primary involvement of the kidney is rare, of the reported cases, 25% of the patients died within 1 year post surgery. Prognosis in the extremities has a better survival rate with an overall 5 year rate of 35-60% with factors that affect that percentage including grade and size of the tumor.

References:
  1. Atkinson, B. Atlas of Diagnostic Cytopathology Second Edition. 2004: 633.
  2. Layfield, L. Cytopathology of the Head and Neck. Johnston, W. ASCP Theory and Practice of Cytopathology 7. 1997: 323.
  3. Keebler, C., Somark, T. The Manual of Cytotechnology 7th Edition. 1993: 317.
  4. Ramzy, I. Clinical Cytopathology and Aspiration Biopsy, Fundamental Principles and Practices 2nd Edition. 2001: 478.
  5. A. Kumar, N. Goyal, S.K. Das, S. Trivedi, U.S. Dwivedi, P.B. Singh: Primary Malignant Fibrous Histiocytoma of the Kidney: Case Report and Review of Literature. The Internet Journal of Surgery, 2007 Volume 12 Number 2.
  6. http://en.wikipedia.org/wiki/Malignant_fibrous_histiocytoma
  7. S. Weiss, F.M. Enzinger: Malignant Fibrous Histiocytoma, An Analysis of 200 Cases. Cancer 41: 2250-2266, 1978.
  8. http://emedicine.medscape.com/article/391453-overview