Practice questions soft tissue tumors

Practice questions

Soft tissue tumors, except skin, cardiac and vascular tumors

©Jun Wang, MD, PhD

1. A 49-year-old woman presents with a soft mass at the upper outer quadrant of her left breast. She has a history of left breast ductal carcinoma in situ 2 years ago and was treated with surgery. She has type II diabetes and has BMI of 31 (normal18.5 to 24.9). Physical examination reveal a 1.5 cm soft mass under unremarkable skin. A 3.5 cm left breast scar is seen consistent with her history of surgery. The mass is resected and grossly it is yellow, soft with lobulated surface, covered by a fine thin capsule. Microscopically it is composed of cells with a large single intracellular clear vacuole and a very small round nucleus with condensed chromatin. No cytological atypia or fibrosis is seen. What is the diagnosis?

A. Lipoma

B. Myxoid liposarcoma

C. Normal mature adipose tissue

D. Recurrent ductal carcinoma in situ

E. Well differentiated liposarcoma

2. Use this case for the next three questions. A 55-year-old postmenopausal woman presents with vague abdominal fullness. She has a history of uterine leiomyoma but declined treatment. She had right oophorectomy 2 years ago due to a mucinous cystadenoma. She had a left forearm melanoma 15 years ago, and was treated with wide excision. Physical examination and her lab tests are unremarkable. Radiological examination reveal a 15 cm retroperitoneal mass. Biopsy of the mass reveal predominantly mature adipose tissue. Scattered atypical cells with dark irregular nuclei and multiple packed cytoplasmic vacuoles. Per immunohistochemistry studies, these cells are positive for vimentin and S-100, but negative for cytokeratin and HMB-45. Mucin stain is negative. What is the most likely diagnosis?

A. Lipoma

B. Metastatic melanoma

C. Metastatic mucinous cystadenocarcinoma

D. Metastatic uterine leiomyoma

E. Well differentiated liposarcoma

3. A 55-year-old postmenopausal woman presents with vague abdominal fullness. She has a history of uterine leiomyoma but declined treatment. She had right oophorectomy 2 years ago due to a mucinous cystadenoma. She had a left forearm melanoma 15 years ago, and was treated with wide excision. Physical examination and her lab tests are unremarkable. Radiological examination reveal a 15 cm retroperitoneal mass. Biopsy of the mass reveal predominantly mature adipose tissue. Scattered atypical cells with dark irregular nuclei and multiple packed cytoplasmic vacuoles. Per immunohistochemistry studies, these cells are positive for vimentin and S-100, but negative for cytokeratin and HMB-45. Mucin stain is negative. What is the most likely genetic abnormality?

A. 12:16 translocation

B. Beta-catenin mutation

C. BRAF mutation

D. Ring/rod chromosome of 12q

E. X:18 translocation

4. A 55-year-old postmenopausal woman presents with vague abdominal fullness. She has a history of uterine leiomyoma but declined treatment. She had right oophorectomy 2 years ago due to a mucinous cystadenoma. She had a left forearm melanoma 15 years ago, and was treated with wide excision. Physical examination and her lab tests are unremarkable. Radiological examination reveal a 15 cm retroperitoneal mass. Biopsy of the mass reveal predominantly mature adipose tissue. Scattered atypical cells with dark irregular nuclei and multiple packed cytoplasmic vacuoles. Per immunohistochemistry studies, these cells are positive for vimentin and S-100, but negative for cytokeratin and HMB-45. Mucin stain is negative.

The tumor was resected. Microscopic examination reveal an area composed of marked pleomorphic cells immediately adjust to tissue with identical morphology seen in the biopsy. These pleomorphic cells are positive for vimentin and S-100, but negative for HMB-45, cytokeratin and CD45. What is the most likely diagnosis?

A. Dedifferentiated liposarcoma

B. Diffuse large B cell lymphoma

C. Malignant transformation of metastatic mucinous cystadenoma

D. Myxoid/round cell liposarcoma

E. Well differentiated liposarcoma

5. Use this case for the next two questions. A 35-year-old man presents with left thigh enlargement for 1 months. His past history is unremarkable. Physical examination reveals a markedly enlarged left thigh without distinct mass. Sonographic examination reveal a 10 cm posterior mass. The mass was resected. Microscopic examination reveal sheets of intermediate sized cells with slightly irregular round nuclei. Scattered adipose cells are seen. Immunohistochemitry studies reveal these intermediate sized cells are positive for vimentin and S-100, but negative for cytokeratin, CD99 and CD45. What is the most likely diagnosis?

A. Burkitt lymphoma

B. Diffuse large B cell lymphoma

C. Ewing sarcoma

D. Lipoma

E. Myxoid/round cell liposarcoma

6. A 35-year-old man presents with left thigh enlargement for 1 months. His past history is unremarkable. Physical examination reveals a markedly enlarged left thigh without distinct mass. Sonographic examination reveal a 10 cm posterior mass. The mass was resected. Microscopic examination reveal sheets of intermediate sized cells with slightly irregular round nuclei. Scattered adipose cells are seen. Immunohistochemitry studies reveal these intermediated cells are positive for vimentin and S-100, but negative for cytokeratin, CD99 and CD45. What genetic abnormality is the most likely seen?

A. t(2;8) involving PAX3 gene

B. t(11,22) involving EWS gene

C. t(12;16) involving FUS gene

D. t(17;22) involving fusion of MYH9 gene

E. t(X;18) involving SYT

7. A 68-year-old woman presents with rapidly increased abdomen distension for a month. She has a history of mucinous cystadenocarcinoma that was treated with surgery and chemotherapy. Physical examination reveals a bulging abdomen with scar consistent with previous surgery. No mass is found. There is no sign of ascites. Her lab tests are unremarkable. CEA is with normal range. Radiological examinations reveal a 17 cm retroperitoneal mass. The mass was resected and microscopic reveals diffuse infiltrated of markedly atypical cells containing nuclei with largely variable sizes and shapes. A few mature adipose cells are seen. There are cells with slightly irregular nuclei and multiple intracellular vacuoles of various sizes. Immunohistochemistry reveal diffuse reactivity to vimentin and S-100. No reactivity to cytokeratin, CD48, HMB45, CEA or desmin is detected. What is the most likely diagnosis?

A. Diffuse large B cell lymphoma

B. Malignant nerve sheath tumor

C. Metastatic ovarian mucinous cystadenocarcinoma

D. Pleomorphic liposarcoma

E. Undifferentiated pleomorphic sarcoma

8. A 23-year-old man presents with a rapidly growing firm mass at his right upper arm. He was hit at the right upper arm during a baseball game that resulted a bruise, but no other complications occurred. Physical examination reveals a 3 cm firm lobulated mass at the right lateral arm. A biopsy was performed and reveals loose connective tissue with myxoid background. There are spindle cells and star-shaped cells among scattered collagen bundles. There are a few red blood cells in the stroma, but no significant inflammation is seen. There is no evidence of significant cytological atypia nor necrosis. What is most likely the diagnosis?

A. Hematoma with regenerative changes

B. Hodgkin lymphoma, lymphocyte depleted type

C. Myxoid liposarcoma

D. Nodular fasciitis

E. Synovial sarcoma

9. A 66-year-old man presents with a firm nodule at his left palm and reduced motion of his index and middle fingers. Physical examination confirmed the presence of 1.5 cm firm nodule located in the radial side of his left palm, appeared to be attached to the flexor tendons. The nodule was resected and microscopic examination reveal slightly hypercellular dense connective tissue. No cytological atypia is seen. Immnohistochemistry studies reveal positive reactivity to vimentin. These cells are negative for desmin, cytokeratin and S-100. What is the most likely diagnosis?

A. Fibrosarcoma

B. Leiomyoma

C. Leiomyosarcoma

D. Malignant peripheral nerve sheath tumor

E. Superficial fibromatosis

10. Use this case for the next three questions. A-21-year old white woman presents with recurrent diarrhea containing blood and mucus for 2 years. She has a family history of multiple colonic cancers at age 20-30 in his father side family members, including her father, who was diagnosed with colon adenocarcinoma at age of 32. Her laboratory tests reveals a hemoglobin at 9 g/dL (normal 12-16 g/dL). White cells and platelets are normal in quantity and morphology. Microbiology studies of stool are repeatedly negative for microorganism. Endoscopic examinations reveal numerous small polyps. Microscopic examination of these polyps reveal straight glands lined by glandular cells with basally located elongated hyperchromic nuclei. Colectomy was performed and during the surgery, a 3 cm firm mass is found attached to the mesentery. Microscopic examination of the mass reveal moderate amount of spindle cells in a largely fibrous background. Immunohistochemistry studies are positive for vimentin, but negative for cytokeratin, desmin and S-100. What is the most likely diagnosis concerning the mesentery firm mass?

A. Deep fibromatosis

B. Fibrosarcoma

C. Leiomyoma

D. Leiomyosarcoma

E. Metastatic adenocarcinoma of colon

11. A-21-year old white woman presents with recurrent diarrhea containing blood and mucus for 2 years. She has a family history of multiple colonic cancers at age 20-30 in his father side family members, including her father, who was diagnosed with colon adenocarcinoma at age of 32. Her laboratory tests reveals a hemoglobin at 9 g/dL (normal 12-16 g/dL). White cells and platelets are normal in quantity and morphology. Microbiology studies of stool are repeatedly negative for microorganism. Endoscopic examinations reveal numerous small polyps. Microscopic examination of these polyps reveal straight glands lined by glandular cells with basally located elongated hyperchromic nuclei. Colectomy was performed and during the surgery, a 3 cm firm mass is found attached to the mesentery. Microscopic examination of the mass reveal moderate amount of spindle cells in a largely fibrous background. Immunohistochemistry studies are positive for vimentin, but negative for cytokeratin, desmin and S-100. Abnormality of what gene does the patient most likely have?

A. Beta-catenin

B. NF1

C. N-myc

D. PAX3

E. SYT

12. A-21-year old white woman presents with recurrent diarrhea containing blood and mucus for 2 years. She has a family history of multiple colonic cancers at age 20-30 in his father side family members, including her father, who was diagnosed with colon adenocarcinoma at age of 32. Her laboratory tests reveals a hemoglobin at 9 g/dL (normal 12-16 g/dL). White cells and platelets are normal in quantity and morphology. Microbiology studies of stool are repeatedly negative for microorganism. Endoscopic examinations reveal numerous small polyps. Microscopic examination of these polyps reveal straight glands lined by glandular cells with basally located elongated hyperchromic nuclei. Colectomy was performed and during the surgery, a 3 cm firm mass is found attached to the mesentery. Microscopic examination of the mass reveal moderate amount of spindle cells in a largely fibrous background. Immunohistochemistry studies are positive for vimentin, but negative for cytokeratin, desmin and S-100. What is the most likely genetic background of this patient?

A. Gardner syndrome

B. Li-Fraumeni syndrome

C. Maffucci syndrome

D. Neurofibromatosis

E. Tuberous sclerosis

13. A 59-yer-old woman presents with a slowly growing mass at her left breast. She has history of poorly differentiated invasive ductal carcinoma of left breast 5 years ago and was treated with surgery, chemotherapy and radiation therapy. Physical examination reveals a 3.5 cm firm mass besides the unremarkable scar of previous left breast surgery. Biopsy reveals hypercellular spindle cell growth with a V-shaped weaving pattern. Scattered mitosis are seen. These cells are positive for vimentin but negative for cytokeratin, S-100, CD34 and desmin. What is the most likely diagnosis?

A. Angiosarcoma

B. Fibrosarcoma

C. Leiomyosarcoma

D. Malignant peripheral nerve sheath tumor

E. Recurrent poorly differentiated invasive ductal carcinoma

14. Use this case for the next two questions. A 5-year-old boy from Central Africa presents with a painful mass on his right face. Physical examination reveal a 6.5 cm firm mass underneath skin, causing marked facial asymmetry. Biopsy of the mass reveal sheets of small to intermediate sized cells with round to oval nuclei and scant to moderate amount of cytoplasm. Numerous mitosis are seen. A few large round cells have abundant eosinophilic cytoplasm. These cells are positive for vimentin, desmin, but negative for CD45, cytokeratin and S-100. What is the most likely diagnosis?

A. Burkitt lymphoma

B. Embryonal rhabdomyosarcoma

C. Leiomyosarcoma

D. Small cell carcinoma

E. Synovial sarcoma

15. A 5-year-old boy from Central Africa presents with a painful mass on his right face. Physical examination reveal a 6.5 cm firm mass underneath skin, causing marked facial asymmetry. Biopsy of the mass reveal sheets of small to intermediate sized cells with round to oval nuclei and scant to moderate amount of cytoplasm. Numerous mitosis are seen. A few large round cells have abundant eosinophilic cytoplasm. These cells are positive for vimentin, desmin, but negative for CD45, cytokeratin and S-100. Abnormality of what gene does the patient most likely have?

A. Beta-catenin

B. NF1

C. N-myc

D. PAX3

E. SYT

16. A 3-year-old girl presents with a lobulated mass in her nasal cavity. Physical examination reveal a polypoid firm mass measuring 2 cm in greatest dimension. Microscopic examination reveals dense small to intermediate sized cells underneath unremarkable epithelium. These cells have eccentric nuclei and eosinophilic cytoplasm. What marker is most likely be positive for these cells?

A. CD38

B. Chromogranin

C. Desmin

D. EMA

E. p16

17. Use this case for the next two questions. A 25-year-old man presents with worsening cough for a month. He had an infectious mononucleosis at age 18, and his past medical/surgical history was otherwise unremarkable. He has been smoking cigarette 1 pack a day for 5 years. Radiologic examinations reveal a 16 cm mediastinum mass extending to left lung. Biopsy of the mass reveal nests of small to intermediate sized cells separated by thick collagen bands. Most of these cells have round to oval nuclei and scant to moderate amount of cytoplasm. A few large cells have centrally located abundant eosinophilic cytoplasm, surrounded by multiple nuclei at periphery. These cells are positive for vimentin, desmin, but negative for CD45, CD20, cytokeratin and S-100. What is the most likely diagnosis?

A. Adult T cell lymphoma

B. Alveolar rhabomyosarcoma

C. Classic Hodgkin lymphoma, nodular sclerosis type

D. Small cell carcinoma

E. Small lymphocytic lymphoma

18. A 25-year-old man presents with worsening cough for a month. He had an infectious mononucleosis at age 18, and his past medical/surgical history was otherwise unremarkable. He has been smoking cigarette 1 pack a day for 5 years. Radiologic examinations reveal a 16 cm mediastinum mass extending to left lung. Biopsy of the mass reveal nests of small to intermediate sized cells separated by thick collagen bands. A few large cells have centrally located abundant eosinophilic cytoplasm, surrounded by multiple nuclei at periphery. These cells are positive for vimentin, desmin, but negative for CD45, cytokeratin and S-100. Abnormality of what gene does the patient most likely have?

A. Beta-catenin

B. EGFR

C. NF1

D. N-myc

E. SYT

19. A 59-year-old postmenopausal woman present with vague lower abdomen discomfort and irregular uterine bleeding for 4 months. Physical examination reveals an enlarged uterus, but otherwise unremarkable. Sonographic examination reveals a 7 cm uterine mass. Hysterectomy was performed and reveals a 7 cm well circumference firm mass at the posterior myometrium. Microscopic examination reveals atrophic endometrium. The myometrium mass is composed of cells with markedly variable sizes and nuclear shapes. Numerous mitosis are seen. These cells are positive for vimentin, desmin, but negative for CD45, cytokeratin and S-100. What is the most likely diagnosis?

A. Diffuse large B cell lymphoma

B. Endometrial stroma sarcoma

C. Leiomyoma with traumatic changes

D. Leiomyosarcoma

E. Malignant mixed mullerian tumor

20. Use this case for the next two questions. A 25-year-old man presents with a slowly growing mass in his right thigh. Physical examination reveal a 3 cm movable firm mass, located at anterolateral portion of his right thigh, 2.5 cm above the knee. Radiologic studies reveal a solid mass detached from femur. The mass was resected. Microscopic examination reveal a growth of spindle cells with scant to moderate amount of cytoplasm. Scattered mitosis are seen. These cells are positive for vimentin, EMA, but negative for CD45, desmin and S-100. What is the most likely diagnosis?

A. Ewing sarcoma

B. Nodular fasciitis

C. Osteosarcoma

D. Rhabdomyosarcoma

E. Synovial sarcoma

21. A 25-year-old man presents with a slowly growing mass in his right thigh. Physical examination reveal a 3 cm movable firm mass, located at anterolateral portion of his right thigh, 2.5 cm above the knee. Radiologic studies reveal a solid mass detached from femur. The mass was resected. Microscopic examination reveal a growth of spindle cells with scant to moderate amount of cytoplasm. Scattered mitosis are seen. These cells are positive for vimentin, EMA, but negative for CD45, demin and S-100. Abnormality of what gene does the patient most likely have?

A. EWS

B. FUS

C. MDM2

D. MYH9

E. SYT

22. Use this case for the next two questions. A 19-year-old man presents with a rapidly growing mass at his forearm. He has a history of multiple neurofibromas. Physical examination reveals multiple light brown areas on his trunk. A few brown dome-shaped growing in his iris. The forearm nodule appears to be firm and fixed to deep tissue. Radiologic examination reveals a 4.5 cm homogeneous mass infiltrating into peripheral tissue. Biopsy reveal a growth of primarily hyperchromic spindle cells with curved nuclei. Which marker is most likely positive for this mass?

A. CD30

B. Desmin

C. EMA

D. S-100

E. von Willebrand's factor

23. A 19-year-old man presents with a rapidly growing mass at his forearm. He has a history of multiple neurofibromas. Physical examination reveals multiple light brown areas on his trunk. A few brown dome-shaped growing in his iris. The forearm nodule appears to be firm and fixed to deep tissue. Radiologic examination reveals a 4.5 cm homogeneous mass infiltrating into peripheral tissue. Biopsy reveal a growth of primarily hyperchromic spindle cells with curved nuclei. Abnormality of what gene is most likely associated with his presentations?

A. Beta-catenin

B. FUS

C. NF1

D. N-Myc

E. SYT

24. A 45-year-old woman presents with multiple scalp nodules for 2 months. She has a history of splenic marginal zone lymphoma that was treated with splenectomy and chemotherapy with rituximab. Physical examination is unremarkable except the few firm scalp nodules up to 1.5 cm in greatest dimension. A diagnosis of sebaceous cysts was made and all these nodules were resected. Microscopic examination of these nodules reveals sheets of markedly atypical large cells with pleomorphic nuclei. These cells are positive for vimentin, but negative for cytokeratin, CD45, desmin, and S-100. What is the most likely diagnosis?

A. Cutaneous T cell lymphoma

B. Diffuse large B cell lymphoma

C. Monomorphic synovial sarcoma

D. Poorly differentiated carcinoma

E. Undifferentiated pleomorphic sarcoma

Answers
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