Page 19 - Delaware Medical Journal - October 2017
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CASE REPORT
was resected and diagnosed as MPNST. A lesion of her left upper extremity was also biopsied, subsequently resected, and diagnosed as MPNST. She then underwent another course of chemotherapy.
A few months later, the patient returned with a palpable mass in the upper-
outer quadrant of her left breast which ultrasound-guided biopsy revealed atypical spindle cells. She subsequently underwent excision by segmental mastectomy. The tumor measured 3
x 2 x 1.9 cm. Histiologic examination showed proliferation of spindle cells Immunohistochemical staining revealed the tumor to be negative for cytokeratin and focally positive for S-100 and CD99, malignant sarcoma. The lesion was favored to be a MPNST based on the patient’s prior history. Furthermore,
the breast tumor was favored to be a primary lesion because there did not appear to be an intravascular component and because the tumor presented with histologic features different from the primary MPNST. While there were small peripheral nerves adjacent to
the lesion, there was no nerve seen in direct association with the tumor. Even though a metastasis from the patient’s pre-existing MPNST is unlikely, it could not be entirely ruled out.
A thoracic surgical oncologist deemed
that there was no role resection of the lung nodules since her disease was multicentric. A thorough literature review revealed no other cases of MPNST of the breast in a
and multiple primaries. Given the patient’s metastatic disease, her prognosis is guarded.
DISCUSSION
MPNST of the breast has been reported in the literature ten other times. Of those, one patient presented with a known varied from cystic-like lesions, to Fine needle aspiration was done in
most cases and in one case, initially thought to be malignant phylloides staining strongly positive for S-100
on immunohistochemistry. Treatment varied, including lumpectomy, wide excision, simple and total mastectomy. There have been no reported cases of nodal involvement. The role of radiation therapy is unknown. Adjuvant radiation was used in four of the reported cases. The only reported use of chemotherapy was by Hauser et al. in a patient with sarcomatous pleuritic.4 Since most soft tissue sarcomas rarely metastasize via lymphatics, resection without lymph node sampling appears to be effective
for isolated breast cases.5 There were no reports of local recurrence on follow up.
CONCLUSION
Sarcomas make up 0.2-1 percent of breast malignancies, and of those, MPNST
has only been reported ten times in the literature.5 To the best of our knowledge, NF-1 and concurrent multiple primary of breast MPNST as a possible new primary. There have been no reports of recurrence after surgical excision with
or without radiation therapy. Given our patient’s complicated course and
metastatic disease, her treatment options are limited. There is scant data on the success of systemic therapy.
CONTRIBUTING AUTHORS
■ ELIANNE ROJAS, DO is a General Surgery Resident at Christiana Care Health System in Newark, Del.
■ JAMIL KHATRI, MD is a Hematologist and Oncologist at Christiana Care Health System in Newark, Del.
■ DIANA DICKSON-WITMER, MD, FACS is Director of the Breast Program at Helen
F. Graham Cancer Center and Research Institute at Christiana Care Health System in Newark, Del.
■ DENNIS WITMER, MD, FACS is a General Surgeon and Vice Chair for Quality and Performance Improvement at Christiana Care Health System in Newark, Del., and a Clinical Associate Professor of Surgery at Sidney Kimmel Medical College at Jefferson University in Philadelphia.
REFERENCES
1. Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors
in neurofibromatosis. Cancer Res. 2002;62:1573–1577.
2. Stucky CC, Johnson KN, Gray RJ, et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012; 19:878.
3. Farid M, Demicco EG, Garcia R, et al. Malignant peripheral nerve sheath tumors. The Oncologist. 2014;19:193-201.
4. Hauser H, Beham A, Steindorfer P, Schmidt F, Smola MG. Malignant schwannomas
of the breast. Langenbecks Arch Chir. 1995;380:350–353.
5. Cil T, Altintas A, Pasa S, Isikdogan A. Primary spindle cell sarcoma of the breast. Breast Care. 2008;3:197-199.
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