| Abstract|| |
Mixed/biphasic tumors include epithelial-myoepithelial tumors, pleomorphic adenoma, matrix-producing tumors, cutaneous mixed tumors such as chondroid syringoma (CS), malignant mixed tumors (carcinosarcomas), pulmonary blastomas (in lung), and many others. Morphology may show overlap between various mixed tumors. At any particular site, whether these tumors are primary or metastasis from other sites is difficult to determine, more so if primary is unknown or not disclosed. CS is a rare benign mixed/biphasic skin adnexal tumor, considered as cutaneous counterpart of pleomorphic adenoma. Its malignant variant, malignant CS is reported only occasionally. We describe a case of a 51 year old female who presented with multiple masses in both lungs. This case is a very rare example of late metastasis to lungs from a primary malignant CS of thigh.
Keywords: Chondroid syringoma, lung, mixed/biphasic tumors
|How to cite this article:|
Kothiya M, Mittal N, Kumar R, Kane S. Malignant chondroid syringoma of thigh with late metastasis to lung: A very rare case report. Indian J Pathol Microbiol 2017;60:418-20
|How to cite this URL:|
Kothiya M, Mittal N, Kumar R, Kane S. Malignant chondroid syringoma of thigh with late metastasis to lung: A very rare case report. Indian J Pathol Microbiol [serial online] 2017 [cited 2020 Feb 29];60:418-20. Available from: http://www.ijpmonline.org/text.asp?2017/60/3/418/215371
| Introduction|| |
Mixed/biphasic tumors include epithelial-myoepithelial tumors, pleomorphic adenoma, matrix-producing tumors, cutaneous mixed tumors such as chondroid syringoma (CS), and many more., Mixed/biphasic tumors of the lung include carcinosarcomas, epithelial-myoepithelial carcinoma, pleomorphic adenoma, and pulmonary blastoma. We describe a case of a 51 year old female who had multiple masses in both lungs, which on biopsy revealed mixed/biphasic tumor. The list of differential diagnoses was extensive; however, it was the history of the patient which helped us arrive at the correct diagnosis.
| Case Report|| |
Our patient was a 51 year old female who presented with dry cough of 2 months duration. The patient underwent radiological evaluation for the same and was found to have multiple opacities in bilateral lungs [Figure 1]. Clinical diagnosis of lung carcinoma with intralung metastasis or metastasis from other primary sites was suspected. Biopsy was done from one of these masses. Histology showed mixed/biphasic tumor with epithelial cells arranged in cords and nests in abundant chondromyxoid matrix [Figure 2]a. The cells were polygonal with enlarged, hyperchromatic, central to peripherally placed nuclei, and eosinophilic cytoplasm. Based on morphology, differential diagnoses of myoepithelial carcinoma, extraskeletal myxoid chondrosarcoma, rare possibility of pleomorphic adenoma primarily arising from salivary glands in the lung, and metastasis from the malignant mixed tumor were considered.
|Figure 1: Computed tomography scan thorax showing multiple nodules in both lungs|
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|Figure 2: (a) Cords and nests of metastatic tumor cells in lung, ×4; (b) calponin negativity in metastatic tumor cells, ×10|
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On immunohistochemistry, tumor cells were positive for AE1/AE3 and cytokeratin 7 (CK7). They were negative for myoepithelial markers (calponin, CD10, and p63), ruling out myoepithelial carcinoma or its metastasis [Figure 2]b. They were also negative for S-100 and TTF1. Positivity for epithelial markers and negativity for S-100 ruled out extraskeletal myxoid chondrosarcoma. Primary from the breast or female genital tract was also ruled out subsequently based on imaging studies. Thus, the remaining differential diagnoses were primary multiple pleomorphic adenoma of the lung and metastasis from malignant mixed tumor, both being very rare.
Immunohistochemistry cannot differentiate between the above two possibilities. Thus, we reviewed our approach and went back to clinical history of the patient. Clinical notes mentioned a scar on the left thigh, details of which were not mentioned. To find the possible cause, we contacted the patient and came to know that she had undergone wide excision of slow-growing nodular mass over left thigh, 3 years back. The specimen was sent to our hospital from outside which had different registration number. Specimen measured 5.5 cm × 4.5 cm × 1.5 cm. On cut section, a gray-white tumor was identified which measured 3.2 cm × 2.8 cm × 1.5 cm. Overlying skin was stretched due to tumor. Sections studied from the tumor showed epithelial tumor cells arranged in cords, trabeculae, and glandular structures in a myxoid matrix [Figure 3]a. Brisk mitotic activity, including atypical forms, was seen in all the sections examined from the tumor [Figure 3]b. A single focus of 0.2 cm in largest dimension was identified showing bland epithelial cells in cords in chondromyxoid stroma, possibly representing remnant benign CS element [Figure 3]c. The tumor morphology of lung mass and thigh mass was similar. Immunohistochemistry done at that time had showed tumor cells positive for CK7 [Figure 3]d and negative for p63. Thus, the primary tumor had same immunoprofile as that of metastatic lesions.
|Figure 3: (a) Malignant tumor infiltrating into the surrounding tissue, scanner view; (b) hyperchromatic nuclei and atypical mitosis, ×20; (c) residual benign chondroid syringoma element, ×10; (d) cytokeratin 7 positivity in tumor cells, ×10|
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The patient did not take any other treatment and was asymptomatic for 2 years. She came back with complaints of dry cough for which she underwent radiological evaluation and was found to have multiple bilateral lung opacities. Considering these details, diagnosis was established as malignant CS of thigh with late metastasis to lungs.
As the lung masses were unresectable, the patient was offered palliative care, as chemotherapy data for this tumor are sparse.
| Discussion|| |
CS is a mixed tumor of the skin adnexa, first described by Hirsch and Helwig, has eccrine differentiation, and it is reported over a wide age range, average being 50 years., Benign CS is usually found in head and neck region with male predilection, these are usually 1–3 cm in size; however, tumors as large as 10 cm with multiple tumors are also reported., On the contrary, malignant CS is extremely rare (<50 cases till now) and very little information is available for it, mostly in the form of case reports and only one case is reported in thigh.,, Like the index case, malignant CS occurs more frequently on the trunks and extremities. It has female predilection., Histological criteria considered for malignancy are cytologic atypia, necrosis, satellite nodules, infiltrative tumor margins, and infiltration of the deeper structure; the index case had moderate to marked cytologic atypia, brisk mitotic activity, and infiltration into the surrounding tissue.
The treatment of malignant CS is mainly wide surgical excision with very limited role of radiotherapy and/or chemotherapy. With malignant CS, nearly half of the patients are reported to have local recurrence. Lymph node metastasis, distant metastasis most often to lungs followed by brain are also reported. Targeted chemotherapies are under investigation in clinical testing, as a potential treatment option. Although rare, it is a differential diagnosis of mixed tumors and it may pose a diagnostic dilemma particularly at a metastatic site, like the index case.
| Conclusions|| |
Malignant CS, though a rare occurrence, has the potential for distant metastasis even after a long period; hence, the history may not be forthcoming. Patients should be accurately diagnosed as malignant CS and should be kept under regular follow-up to detect metastasis early, which makes them amenable to surgical excision with curative intent. The possibility of metastasis from rare tumors such as malignant CS should also be considered in the differential diagnoses of malignant mixed tumors at various sites.
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Conflicts of interest
There are no conflicts of interest.
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Department of Pathology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]