Two Forensic Autopsy Cases of Liposarcoma

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Case Report

Two Forensic Autopsy Cases of Liposarcoma Kino Hayashi, Kumiko Asakura, Nao Miyashita1, Akihiko Hamamatsu, Akio Shigeta, Tatsushige Fukunaga Tokyo Medical Examiner’s Office, 1Specific Disease Control Section, Bureau of Social Welfare and Public Health, Tokyo Metropolitan Government, Tokyo, Japan

Abstract Primary liposarcoma outside thighs is rare. We present two forensic autopsy cases of clinically undiagnosed primary liposarcoma (Case 1: Mediastinum, Case 2: Mesentery). Case 1 was a thin male in his 60s who had a well‑differentiated liposarcoma (850 g, 23 cm × 20 cm × 10 cm) in the superior mediastinum. Case 2 was a thin female in her 50s who had a pleomorphic liposarcoma (10 kg, 40 cm × 25 cm × 11 cm) in the abdominal cavity with liver metastases. The cause of death in Case 1 was airway obstruction due to liposarcoma of the neck; that in Case 2 was complications of liposarcoma with metastasis. Because liposarcoma often appears in deeper tissue without pain, it will grow quite large before it is noticed. Key words: Forensic autopsy case, mediastinal liposarcoma, mesenteric liposarcoma

Introduction Liposarcoma can occur anywhere in the body, but more than 40% of liposarcoma cases involve the lower extremities, especially the thigh.[1] Liposarcoma is the second most common soft tissue sarcoma in adults with a peak incidence between 40 and 60 years and slight preponderance to male gender.[1‑3] Liposarcoma can often grow into large tumors because it appears in deeper tissue without pain.[3] This paper presents two middle‑aged autopsy cases of clinically undiagnosed primary liposarcoma, which originated from the mediastinum in Case 1 and the mesentery in Case 2.

Case Reports Case 1

A thin male in his 60s who worked as a part‑time driver had a swelling around the neck and a sputum‑producing cough with stroke like a transient cerebral ischemic attack (TIA) for a year. However, he did not like doctor and medical treatment, so he did not go to hospital. He was a person who smoked and used alcohol daily (500 ml of beer a day). He had a stroke suddenly at home after severe coughing and passed away in spite of first‑aid treatment at the emergency hospital. According to the doctor of the emergency hospital, it was difficult to open the airway because of the tumor in the neck. The deceased was 171 cm tall and weighed 60 kg, with a body mass index of 20.5 kg/m2. His neck showed symmetrical, massive expansion [Figure 1a]. An autopsy revealed a large yellowish well‑circumscribed Access this article online Quick Response Code:

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DOI: 10.4103/2349-5014.191473

tumor (850 g, 23 cm × 20 cm × 10 cm in size) in the superior mediastinum. The tumor could be easily separated from the other organs [Figure 1b and c, Figure 2a and b]. Figure 2c showed the cross sections of the tumor, which compresses the esophagus and the trachea. Figure 3a showed the tumor with the esophagus and the trachea in the superior mediastinum with a macroscopic view. The tumor composed lipoblasts and broad fibrous septa [Figure 3b]. There was a lot of lipoblasts with atypical hyperchromatic nuclei [Figure 3c and d]. This tumor was diagnosed as a well‑differentiated liposarcoma. The heart was weighed 500 g without severe coronary stenosis and ischemic change. The brain was weighed 1493 g without infarction. From the symptom of a TIA with a sputum‑producing cough and acute sudden death, the cause of death was diagnosed as the airway obstruction due to liposarcoma of the neck.

Case 2

A thin female in her 50s was found dead in her room. She could not work because of extreme weight loss without medical care for a few months prior to the death. She was a person who smoked and drank little alcohol. The

Address for correspondence: Dr. Kino Hayashi, Tokyo Medical Examiner’s Office, Tokyo, Japan. E‑Mail: kino‑[email protected]

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How to cite this article: Hayashi K, Asakura K, Miyashita N, Hamamatsu A, Shigeta A, Fukunaga T. Two Forensic Autopsy Cases of Liposarcoma. J Forensic Sci Med 2016;2:175-8.

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Figure 1: Gross appearance of Case 1. (a) He had a swelling around the neck. (b) The tumor attached to the thyroid gland with clear border. (c) The tumor could be easily separated from the other organs

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Figure 2: Macroscopic examinations of the liposarcoma, Case 1. A large yellowish well‑circumscribed tumor (850 g, 23 cm × 20 cm × 10 cm). (a) Posterior side of the tumor. (b) Anterior side of the tumor. (c) Cross sections of the tumor, which compresses the esophagus and the trachea

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Figure 3: Microscopic findings of the liposarcoma, Case 1. (a) Well‑differentiated liposarcoma with the esophagus and the trachea in the superior mediastinum with macroscopic view (H and E stain, magnified view of a white square in Figure 2c). (b) It composed lipoblasts and broad fibrous septa (H and E stain, magnified view of a white square in Figure 3a). (c and d) Lipoblasts with atypical hyperchromatic nuclei (H and E stain, c: Magnified view of a white square in Figure 3b, d: Magnified view of a white square in Figure 3c)

deceased was 170 cm tall and weighed 52 kg, with a body mass index of 18.0 kg/m 2. She had an enormous abdominal swelling [Figure 4a] with severe leg edema. An autopsy revealed a large reddish‑brown well‑circumscribed tumor (approximately 10 kg, 40 cm × 25 cm × 11 cm in size) in the abdominal cavity [Figure 4b]. This tumor seemed to originate from the mesentery of the transverse or ascending colon and could be easily separated from the intestines and pelvic organs [Figure 5a‑d]. The cross‑section of the tumor showed whitish solid and reddish polycystic areas [Figure 5e and f]. Microscopic examination of the primary tumor revealed fibrous tissue and pleomorphic lipoblasts [Figure 6a‑d]. The lipoblasts were negative for S100 protein, MDM2, and CDK4. There 176

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Figure 4: Gross appearance of Case 2. (a) She presented with abdominal distention. (b) A large tumor in the abdominal cavity. The tumor seemed to originate from the mesentery and could be easily separated from the other organs

was massive necrosis without well‑differentiated liposarcoma portion [Figure 6a]. This tumor was diagnosed as a pleomorphic liposarcoma. Liver metastasis (1 cm in diameter) was also found [Figure 7a and b]. Systemic fatty tissue showed serous atrophy and the blood total ketone bodies were high (1240 µmol/l). The autopsy findings showed that she had starved for a long time. The cause of death was diagnosed as complications of liposarcoma with metastasis.

Discussion The 2013 WHO classification of soft tissue tumors[2] was revised for the first time in about a decade. This newly‑revised classification recognizes five categories of liposarcoma: (1) Well‑differentiated liposarcomas are most common and low‑grade malignancy that recur but do not metastasize. (2) Dedifferentiated liposarcomas are characterized by the coexistence of well differentiated and poorly differentiated.

Journal of Forensic Science and Medicine ¦ July 2016 ¦ Volume 2 ¦ Issue 3

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Figure 5: Macroscopic examinations of the liposarcoma, Case 2. An enormous tumor with solid and polycystic areas (10 kg, 40 cm × 25 cm × 11 cm). (a) The anterior side of the tumor (A: Right side, B: Left side). (b) The anterior side of the tumor. Greenish areas (*) showing the areas adjacent to the transverse colon. (c) The posterior side of the tumor. (d) The posterior side of the tumor. Greenish area (**) showing the area adjacent to the ascending colon. (e) Cross‑section of this tumor (–). (f) Cross‑section of the tumor, showing whitish solid and reddish polycystic areas

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Figure 6: Microscopic findings of the liposarcoma, Case 2. (a) Pleomorphic liposarcoma showing some necrotic lesions (H and E). (b and c) Pleomorphic liposarcoma with multivacuolar lipoblasts (H and E). (d) Pleomorphic liposarcoma (oil red O stain)

Figure 7: Microscopic findings of the liver metastases, Case 2. (a) A liver metastasis (1 cm in diameter) (H and E). (b) Metastatic pleomorphic liposarcoma with clustering lipoblasts (H and E, magnified view of a whitish square in Figure 7a)

(3) Myxoid liposarcomas are second common and composed of three main tissue components such as (1) proliferating lipoblasts, (2) a delicate plexiform capillary pattern, and (3) a myxoid matrix. (4) Round cell liposarcomas (poorly differentiated myxoid liposarcoma) are related to myxoid liposarcomas; both have the same genetic findings. It deserves separate consideration because round cell liposarcomas have aggressive clinical course and metastatic tendency.

(5) Pleomorphic liposarcomas have a disorderly growth pattern and an extreme degree of cellular pleomorphism. At first, the macroscopic view of the tumor of Case 1 seemed Madelung’s disease because of symmetrical abnormal deposits of adipose tissue in the neck and upper trunk.[4‑7] Madelung’s disease is also known as benign symmetric lipomatosis, multiple symmetric lipomatosis, fatty neck syndrome, or the Launois‑Bensaude syndrome.[7] It is a rare disorder of unknown


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cause; however, up to 90% of patients have a history of chronic alcoholism.[5] He was a person who used alcohol daily, like patients with Madelung’s disease. However, we denied Madelung’s disease because of the evidence of lipoblasts. On the other hand, spindle cell lipoma is also considered in the differential diagnosis of Case 1. Spindle cell lipomas are benign and typically occur in neck and upper trunk in older males.[2] After the microscopic examination, this tumor was diagnosed as malignant because of the massive presence of lipoblasts. Liposarcoma is rare in the mediastinum.[1,2,8] According to the article,[8] among 24 cases of mediastinal liposarcoma, 9 tumors were located in the anterior mediastinum, 7 in the posterior mediastinum, and 1 in the superior mediastinum. Histologic examination revealed that most of the cases were well‑differentiated liposarcomas (10 cases), followed by dedifferentiated liposarcomas (8 cases). Case 1 was a well‑differentiated liposarcoma which located in the superior mediastinum. The retroperitoneum, including mesentery is the second most common site, with 15%–20% of liposarcomas.[1] In Case 2, the tumor of enormous size arose in the mesentery and filled the entire abdominal cavity. Microscopic examination of the primary tumor revealed fibrous and myxoid stroma with marked pleomorphic cells. The lipoblasts of Case 2 were negative for S100 protein, MDM2, and CDK4. In pleomorphic liposarcomas, staining for S100 protein is positive in lipoblasts in fewer than half of cases, and staining for MDM2 and CDK4 is typically negative.[2] S100 protein staining may be negative in highly undifferentiated tumors.[9] Because of the presence of the large and giant cells without well‑differentiated liposarcoma portion, and the result for immunophenotype, the tumor was diagnosed as a pleomorphic liposarcoma. The symptoms of mesentery[9‑13] or omental[14] liposarcomas typically include painless and progressive abdominal distention. Although liposarcomas sometimes weigh as much as 10 kg or more, those exceeding 20 kg are rare.[10] According to the articles, the heaviest tumor was 46.6 kg.[11] In Japan, the heaviest liposarcoma was 22 kg.[10] We think the only symptom was leg edema produced by the compression on the inferior vena cava by the tumor and that was why she did not go to a hospital and left untreated.

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We have reported two rare cases of clinically undiagnosed primary liposarcoma. Liposarcoma can occur anywhere in the body, so we have to consider liposarcoma as one of the differential diagnoses when we saw a giant tumor of unknown primary in forensic autopsy.

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Conflicts of interest

There are no conflicts of interest.

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