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Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Jennifer Bynum
Clinical history: A 50 year-old female presents with a lung mass
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Question 1 of 1
1. Question
Week 635: Case 1
A 50 year-old female presents with a lung mass
Correct
Answer: Metastatic pleomorphic sarcoma, consistent with known uterine leiomyosarcoma
Histology: The specimen shows a wedge resection of lung with alveolated lung parenchyma and a discrete, fairly well circumscribed parenchymal nodule with no associated desmoplasia or reactive changes. The nodule is comprised of markedly pleomorphic and atypical cells, with variation in nuclear size and shape, hyperchromasia, nucleoli, and brisk mitotic activity including atypical mitotic figures. There is no necrosis. The cells in areas are loosely cohesive and have abundant eosinophilic, glassy cytoplasm. In areas, the cells have a pseudo-vascular growth pattern (i.e., appear to be making slit-like or vascular-like spaces). Immunostains for cytokeratins, smooth muscle markers, skeletal muscle markers, vascular markers, and melanoma markers, are all negative.
Discussion: The patient has a known history of a uterine leiomyosarcoma, however the lesion is in the lung does not display overt histologic or immunophenotypic evidence of smooth muscle differentiation. The slides from the hysterectomy were pulled and re-reviewed, and indeed the tumor in the uterus displayed the classic fascicular growth pattern along with atypia and mitoses of a leiomyosarcoma. However, there were also regions of marked pleomorphism comprised of cells with prominent eosinophilic, glassy cytoplasm. The lesion in the lung is therefore a metastatic pleomorphic sarcoma, consistent with spread from the patient’s uterine leiomyosarcoma. The differential diagnosis of the lung lesion on the basis of the histology should broadly include melanoma, sarcomatoid carcinoma and sarcoma (including angiosarcoma, rhabdomyosarcoma, and pleomorphic undifferentiated sarcoma), with a targeted immunopanel to evaluate any evidence of lineage of differentiation. The most useful component of this case is comparison of the new lung lesion with the patient’s prior hysterectomy slides, and the importance of both clinical history and comparison with prior histology cannot be overstated.
Reference(s):
1. Goldblum JR. An approach to pleomorphic sarcomas: can we subclassify, and does it matter? Mod Pathol. 2014 Jan;27 Suppl 1:S39-46.
2. Oda Y, Miyajima K, Kawaguchi K, et al. Pleomorphic leiomyosarcoma: clinicopathologic and immunohistochemical study with special emphasis on its distinction from ordinary leiomyosarcoma and malignant fibrous histiocytoma. Am J Surg Pathol. 2001 Aug;25(8):1030-8.Incorrect
Answer: Metastatic pleomorphic sarcoma, consistent with known uterine leiomyosarcoma
Histology: The specimen shows a wedge resection of lung with alveolated lung parenchyma and a discrete, fairly well circumscribed parenchymal nodule with no associated desmoplasia or reactive changes. The nodule is comprised of markedly pleomorphic and atypical cells, with variation in nuclear size and shape, hyperchromasia, nucleoli, and brisk mitotic activity including atypical mitotic figures. There is no necrosis. The cells in areas are loosely cohesive and have abundant eosinophilic, glassy cytoplasm. In areas, the cells have a pseudo-vascular growth pattern (i.e., appear to be making slit-like or vascular-like spaces). Immunostains for cytokeratins, smooth muscle markers, skeletal muscle markers, vascular markers, and melanoma markers, are all negative.
Discussion: The patient has a known history of a uterine leiomyosarcoma, however the lesion is in the lung does not display overt histologic or immunophenotypic evidence of smooth muscle differentiation. The slides from the hysterectomy were pulled and re-reviewed, and indeed the tumor in the uterus displayed the classic fascicular growth pattern along with atypia and mitoses of a leiomyosarcoma. However, there were also regions of marked pleomorphism comprised of cells with prominent eosinophilic, glassy cytoplasm. The lesion in the lung is therefore a metastatic pleomorphic sarcoma, consistent with spread from the patient’s uterine leiomyosarcoma. The differential diagnosis of the lung lesion on the basis of the histology should broadly include melanoma, sarcomatoid carcinoma and sarcoma (including angiosarcoma, rhabdomyosarcoma, and pleomorphic undifferentiated sarcoma), with a targeted immunopanel to evaluate any evidence of lineage of differentiation. The most useful component of this case is comparison of the new lung lesion with the patient’s prior hysterectomy slides, and the importance of both clinical history and comparison with prior histology cannot be overstated.
Reference(s):
1. Goldblum JR. An approach to pleomorphic sarcomas: can we subclassify, and does it matter? Mod Pathol. 2014 Jan;27 Suppl 1:S39-46.
2. Oda Y, Miyajima K, Kawaguchi K, et al. Pleomorphic leiomyosarcoma: clinicopathologic and immunohistochemical study with special emphasis on its distinction from ordinary leiomyosarcoma and malignant fibrous histiocytoma. Am J Surg Pathol. 2001 Aug;25(8):1030-8.
