Malignant fibrous histiocytoma of the chest wall: A rare differential.

Dear Editor,

The most common differential diagnosis of a chest wall mass with rib destruction is metastasis (usually from cancers of the breast, lung, kidney, or thyroid) followed by multiple myeloma.

A 70-year-old man, a hypertensive and a diabetic, controlled on medications, presented to us with a painless mass in the right lateral chest wall, of seven months’ duration. The clinical examination revealed a 9 × 8 cm, well-circumscribed mass lesion originating from the fifth, sixth, and seventh ribs, with a large soft tissue component [ Figure 1 ]. A CT scan of the chest confirmed the lesion to be originating mainly from the fifth rib, with involvement of the adjacent fourth and sixth ribs, compressing the adjacent lung parenchyma [ Figure 2 ]. A trucut biopsy from the mass revealed a poorly differentiated tumor, which on immunohistochemistry correlation was suggestive of a high-grade malignant fibrous histiocytoma (MFH) (Immunopositive to vimentin, EMA, and CD 68, negative to keratin, Melan A, and HMB-45. Fifty percent of the tumor cells showed a strong nuclear positivity to Ki-67) [ Figure 3 ]. The patient was taken up for surgery and a radical en-bloc excision of the chest wall tumor was performed, which included the fourth to sixth ribs, along with a wedge of the adjacent lung parenchyma [ Figure 4 ]. The resultant chest wall defect was reconstructed using a Prolene mesh. A decision was made to achieve a skin and soft tissue cover using a local rotation flap, considering his elderly age and the associated comorbid conditions. The final histopathology confirmed the diagnosis of a primary MFH resected with clear margins. The patient was offered, but did not wish to consider the option of adjuvant radiotherapy. He was disease-free for a year, following which he developed right-sided hemorrhagic pleural effusion and opted to be on supportive care.

Malignant fibrous histiocytoma is a deep-seated pleomorphic sarcoma of uncertain origin, occurring in middle-aged adults. It is commonly known to originate in the deep fascia and skeletal muscles of the extremities followed by the trunk and the head and neck.[ 1 ] The involvement of the chest wall is uncommon.[ 2 ]

Surgery in the form of wide excision is the primary modality of management of MFH, including those originating from the chest wall.[ 3 ] Multidisciplinary approaches involving the use of radiotherapy and chemotherapy have been utilized in various combinations, in an attempt to impact the overall survival.[ 4 ] However, the experience with chemotherapy and radiotherapy is insufficient; the number of cases and the reported follow-up is inconsistent to allow conclusions to be drawn as to their effectiveness. MFH has a high propensity for local recurrences (44%) and distant metastasis (42%). The prognostic factors that are known to adversely impact survival in patients with MFH include, a tumor size > 5 cm, deep-seated location, high histological grade, and a high stage, based on the American Joint Committee on Cancer Staging System,[ 5 ] all of which were present in our patient. In conclusion, a primary MFH must to be considered as a differential in a patient with chest wall lesion and rib destruction; such a clinical scenario warrants a radical curative approach.

References

1. Belal A, Kandil A, Allam A, Khafaga Y, El-Husseiny G, El-Enbaby A, et al. Malignant fibrous histiocytoma: A retrospective study of 109 cases. Am J Clin Oncol. 2002;25:16–22. doi: 10.1097/00000421-200202000-00003.

2. Sawai H, Kamiya A, Kurahashi S, Yamanaka Y, Manabe T. Malignant fibrous histiocytoma originating from the chest wall: Report of a case and collective review of cases. Surg Today. 1998;28:459–63. doi: 10.1007/s005950050165.

3. Otsuka T, Harada A, Wakida K, Aoki M, Nagata T, Kariatsumari K, et al. Resection of malignant fibrous histiocytoma through a combined thoracic and abdominal wall approach Kyobu Geka. 2012;65:389–92.

4. Kitazono I, Saigenji H. Long-term survival of malignant fibrous histiocytoma of the chest wall by multidisciplinary treatment. Kyobu Geka. 2007;60:221–4.

5. Oda Y, Tamiya S, Oshiro Y, Hachitanda Y, Kinukawa N, Iwamoto Y, et al. Reassessment and clinicopathological prognostic factors of malignant fibrous histiocytoma of soft parts. Pathol Int. 2002;52:595–606. doi: 10.1046/j.1440-1827.2002.01399.x.