Internal Medicine

Delayed Diagnosis of high grade pleomorphic sarcoma

Comments are accepted only from Internal Medicine experts.

  • 2 Experts requested
  • Case closed
  • 3 Responses

Case Overview

  • FL
  • 67 years old, Male
  • HTN, Cancer, Prior hx of lung cancer
  • Left partial lobectomy lung

***MEDICAL ONCOLOGISTS ONLY***
Please do not leave a comment if you are not a medical oncologist.

Patient had a fall in July 2019 in which he injured his leg and tore his left biceps demoris muscle. He also had lump on his leg.

August 21, 2019
XR showed "No evidence of soft tissue foreign body. No lytic or blastic lesion. No periosteal reaction. There is questionable soft tissue calcification posterior mid thigh. No acute fracture."
He also had a Venous Duplex negative for DVT but with Incidental findings of a large complex cystic mass which appears to be c/w a hematoma [13 x 6.2cm]. No clear Doppler signal inside of mass. Comment: Likely hematoma noted in left upper thigh. Recommendations: Clinical correlation. Consideration for CT for further evaluation."

September 2019
Lump on leg continued to swell and increased pain. He continued seeing doctors ,
CT scan showed "Very large mass in vastus lateralis muscle. 16.6 by 10.2cm by 6.1cm, Largely cystic with multiple enhancing septations and some solid components. Appears to be contained within the muscle. No significant lymphadenopathy."

October 2019
An ortho surgeon recommended draining the hematoma. Evacuation of hematoma done on 10/3/19.

November 2019
Mass increased in size, so they drained it again. From op note: "He had 1L of bloody serous fluid within the deep anterior compartment of the thigh muscle under a significant amount of pressure. The edges of the deep muscle belly had a lot of oozing with an obious tear of the muscle. Looks today with signs of healing such as granulation tissue and a capsule. I did not see any signs of active bleeding."

December 2019
MRI was done. Findings: 14.2 x 313.4 x 33cm. There is no significant increase in size. Interval development of extension to the anterolateral fascia into the subcutaneous tissues laterally, correlate with history of biopsy. Cystic neoplasm must be excluded.

January 2020
Biopsy performed on 1/10/20: Dx: High grade pleomorphic sarcoma suggestive of vascular origin. Tumor cells are positive for FLI1 and CD34, focally positive for desmin, while negative for CD31, ERG, AE1/AE3/CAM5.2, S-100, SOX-10, MDM2, CD68, and CD163. Background histiocytes are highlighted by CD163. These findings in correlation with the patient’s hx of left thigh recurrent hematoma, a sarcoma of vascular origin is suggested.
CT TAP on 1/15/20: 20cm complex mass in thigh with known hx of sarcoma. Left inguinal and pelvic sidewall adenopathy, most concerning for nodal metastasis.
No other highly suspicious sites of metastatic disease in the chest, abdomen, or pelvis. Sub-5 mm left upper lobe pulmonary nodule and subcapsular liver hypodensitites require attention on imaging surveillance.

February 2020
2/14/20 Cancer Center note: Dx: High grade pleomorphic sarcoma. Surgical margin, uninvolved by tumor. Comment: Grossly, a very large (37.3cm) heterogenous mass composed of solid, cystic and necrotic areas is identified. Representative histologic sections demonstrate a pleomorphic, high grade sarcoma containing variable number of pleomorphic lipoblasts adjacent to non-lipogenic areas resembling an undifferentiated sarcoma. There are cystic areas containing blood and fibrin. There are large areas of necrosis (30%). Given the histopmorphologic findings in conjunction with the immunihistochecmical findings, this high grade pleomorphic sarcoma is suggestive of a pleomorphic liposarcoma. Additional studies needed.

Patient is now Stage IV and going through treatment. Questions are whether earlier intervention could have prevented metastasis and whether earlier treaters should have ordered biopsy or ruled out sarcoma.

Files:

Case Questions

No questions yet!

3 Case Responses - Was there any negligence?

Do you believe there might have been medical error?

0 10
3 - Very Unlikely

The patient presented in August 13 cm cystic mass which grew to about 14.2 cm in size. Even, if she was diagnosed in September 2019, her outcome would be the same due to the high-grade nature of Pleomorphic sarcoma.

Do you believe there might have been causation (i.e. the medical error resulted in an injury)?

0 10
3 - Very Unlikely

In January 2020, surgical resection showed margin free from tumor. So there is no causation. it is the natural progression of the disease.

What makes you a good expert for this case?

I have done work on both the plaintiffs and the defense side. it is difficult to pinpoint that size will make a difference as we look into the biology of the tumor which is high grade making her risk for recurrence high.

How often do you encounter cases similar to this one in your practice?

I do see a fair number of soft tissue sarcoma. I have 12 patients currently undergoing treatment.

Do you believe there might have been medical error?

0 10
7 - Likely

The diagnosis of hematoma was suggested in the initial August 2019 XRay as possible Hematoma. The CT scan on September 2019: Very large mass in vastus lateralis muscle. 16.6 by 10.2cm by 6.1cm, Largely cystic with multiple enhancing septations and some solid components. Appears to be contained within the muscle. No significant lymphadenopathy." At that time an exploration and excision of the mass might have offered a better prognosis. The Pleomorphic Liposarcomas are rare and associated with high-grade malignancy, with a high recurrence rate and poor prognosis. They are aggressive and spread rapidly. Additionally the larger the tumor size (>10 cm),or with necrosis present, the worse the prognosis, as evident by more frequent relapses, and dramatically decreased survival time. MRI data is recommended as was done in December 2019 elucidating:: "MRI was done. Findings: 14.2 x 313.4 x 33cm. There is no significant increase in size. Interval development of extension to the anterolateral fascia into the subcutaneous tissues laterally, correlate with history of biopsy. Cystic neoplasm must be excluded." The delay with associated multiple surgical drainage procedures may have breached the vasculature rresulting in the rapid metastatic spread. as noted the tumor after the first "hematoma evacuation" rapidly grew back. Pleomorphic Lipsarcoma (LPS) are very difficult to treat. Data from chemotherpay remains limited. Some dely in progression has been noted with Radiotherapy. But not a curative measure.

Do you believe there might have been causation (i.e. the medical error resulted in an injury)?

0 10
7 - Likely

Delay in diagnosis with multiple non-curative surgical procedures. The initial thought of hematoma and directed care towards its evacuation even after the rapid recurrence should have alerted the physician to something more sinister such as an aggressive neoplasm. Appropritae measures in the initial instance might have prolonged survival. Given the aggressive nature of the malignancy, breaching the vascular tree via surgical intervention in aggressive malignancies has been shown cellular spread. Removal of the entire tumor within its walls may have benefitted the patient.

What makes you a good expert for this case?

Medical Oncologist and a Cell Biologist who can ascertain cell division in between the growth and the noted spread would make agood expert witness.

How often do you encounter cases similar to this one in your practice?

It is very rare and I have very litle experience with a PLS.

Do you believe there might have been medical error?

0 10
8 - Very Likely

Sarcoma can present wtih bleeding. When the hematoma did not quickly resolve, a malignancy needed to be considered and could have been diagnosed with an MRI in October 2019. This means at least 4 months delay in diagnosis. Prognosis is 77% at 5 years for patients with tumors arising on the trunk and extremities. The childhood variant appears have better prognosis. Now that it is metastatic, prognosis is that he will eventually succumb to this disease.

Do you believe there might have been causation (i.e. the medical error resulted in an injury)?

0 10
8 - Very Likely

Prognosis is 77% at 5 years for patients with tumors arising on the trunk and extremities. The childhood variant appears have better prognosis. Now that it is metastatic, prognosis is that he will eventually succumb to this disease.

What makes you a good expert for this case?

The Hematologist/Oncologist who wrote this review had been Associate Professor of Medicine while a full-time attending at the University Hospital of a Medical School until 2009. Prior to 2004 had been an Associate Clinical Professor of Medicine. He is currently in private practice. He is first author of over thirty academic articles, chapters and several books. Over the past two decades he held the positions of Interim Chief of Hematology and Oncology at a Medical School, Director of the Cancer Center at a teaching community hospital; Chief of Hematology and Oncology and Chief of Service at a large municipal Medical Center and concurrently Director of the Cancer Center of several hospital in a network; and Co-Director of Oncology at an affiliated University Hospital and Medical Center. He developed and ran two clinical research programs as well as a community advocacy group, a consulting group, and a non-profit educational institution. In addition to Internal Medicine and Oncology, the reviewer is Board Certified in Quality Assurance and Utilization Review and holds an MBA. He was listed several times as the best in his specialty by the Castle Connolly Guide to Americas top Doctors.

How often do you encounter cases similar to this one in your practice?

At one time, I worked at an institution with a Sarcoma service and saw one about every two weeks. Now - about 6 a year.