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Clinics of Surgery
Case Report ISSN: 2638-1451 Volume 7
Rare Case Of Primary Pulmonary Dedifferentiated Liposarcoma
Tuoro A¹*
, Aversa V² and Cassina EM¹
¹Thoracic Surgery Unit, “San Gerardo” Hospital, Monza, Italy
²University “Magna Graecia” of Catanzaro, Catanzaro, Italy
*
Corresponding author:
Antonio Tuoro,
Thoracic Surgery Unit, “San Gerardo” Hospital,
Monza, Italy, E-mail: aversa.vincenzo@hotmail.it
Received: 31 Mar 2022
Accepted: 12 Apr 2022
Published: 18 Apr 2022
J Short Name: COS
Copyright:
©2022 Tuoro A, This is an open access article distribut-
ed under the terms of the Creative Commons Attribution
License, which permits unrestricted use, distribution, and
build upon your work non-commercially.
Citation:
Tuoro A, Rare Case Of Primary Pulmonary Dedifferentiat-
ed Liposarcoma. Clin Surg. V7(8): 1-5
clinicsofsurgery.com 1
1. Abstract
Liposarcoma is a relatively common soft tissue sarcoma, but pri-
mary pulmonary liposarcomas are extremely rare, expecially for
dedifferentiated subtype. We report the case of a 44‑year‑old Af-
rican woman, healed from SARS-Covid, who presented with a 8
cm right lung ilary mass and underwent to a right intrapericar-
dial pneumonectomy with diaphragmatic and pleural resections.
Histological test confirmed diagnosis of dedifferentiated primary
pulmonary liposarcoma. The patient recovered well having an un-
complicated postoperative course and was discharged after 7 days.
On four months follow up the patient was still alive and without
evidence of disease. More studies have to be conducted because
very few cases are reported in literature.
2. Introduction
Primary pulmonary liposarcoma is an extremely rare malignancy
with very few cases reported in literature. Intrathoracic liposar-
comas are uncommon, and generally located in the mediastinum.
Malignant degeneration of a pulmonary lipoma and pleuropulmo-
nary asbestosis have been considered as possible pathogenetic fac-
tors [1]. Of all subtypes, dedifferentiated is the most uncommon
with only four cases descripted in literature.
3. Case Presentation
A 44-year-old moroccan woman arrived to our Hospital because
of recurring hemoptysis. She underwent about a month before to
a Torax-TC that exposed a right lung ilary mass (7,2 cm) treated
with antibiotic. An EBUS was performed without bioptic sample
because of suspected Echinococcus disease. Once excluded, a
transthoracic biopsy was performed, but samples were not clin-
ically adeqate because of nectotic tissue. During hospitalization
she developed pain associated with hyperpyrexia and increased in-
flammation indices, treated with analgesic and antibiotic therapy.
A Body-TC [Figure 1, 2] exposed an increment of known mass (8
cm), and body-PET [Figure 2] reported an uneven captation, in
particular at the origin of upper right bronchus (SUVmax = 5.84),
at middle right bronchus (SUVmax = 6.09) and at middle right
bronchus biforcation (SUVmax = 6.03) and no other place worth
of notice. So it was decided to perform a right thoracotomy in or-
der to reach the mass. Extemporaneous histological test confirmed
malignity of the mass, so it was dedided to perform a right pneu-
monectomy with diaphragmatic and pleural resections due to infil-
tration [Figure 4, 5]. Bronchial stump was covered by intercostal
muscle previously isolated. A 32 CH thoracic dreinage was placed.
Postoperative period was uneventful, drainage was removed after
2 days and the patient was discharged after 7 days asynthomatic
and in good general conditions. On 10 days follow up the patient
was still asynthomatic with normal outcomes of pneumonectomy.
Histological test confirmed for primary pulmonary liposarcoma
dedifferentiated with S100+ and MDM2.
clinicsofsurgery.com 2
Volume 7 Issue 8 -2022 Case Report
Figure 1: body-TC showing large mass uneven capitation.
Figure 2: Thorax-RX.
clinicsofsurgery.com 3
Volume 7 Issue 8 -2022 Case Report
Figure 3: PET showing.
Figure 4
clinicsofsurgery.com 4
Volume 7 Issue 8 -2022 Case Report
Figure 5
4. Discussion
The first case of PPL was reported by Latienda in 1946 [2]. Till
then very few cases have been reported in literature. It originates
from primitive mesenchymal cell and is common in extremities
and retroperitoneum. PPL generally originates in the mediastinum,
rarely arises from parenchyma, chest wall or pleura. Possible risk
factors associated with development of primary pulmonary lipo-
sarcoma are pleuropulmonary asbestosis or malignant transforma-
tion of pulmonary lipoma [3]. In our case the patient had contact
with asbestos because of the house where she lived, but there was
no sign of asbestosis. No particular characteristic symptoms and
signs are attributable to PPL but are the same of any other pul-
monary tumors, such as cough, sputum production, hemoptysis,
dyspnea, chest pain, and loss of weight and appetite. This can lead
to further delays diagnosis. Also, because these tumors are slow
growing, they often present as a large mass [4]. Diagnosis is based
on histological test. The World Health Organization (WHO) clas-
sifies liposarcomas into four subtypes [5]:
• Atypical well-differentiated, probably benign
• Myxoid/round cell, locally aggressive frequently with metas-
tasies
• Dedifferentiated, very aggressive and higly malignant
• Pleomorfic, malignant
In literature we found only four cases of dedifferentiated Primary
pulmonar liposarcoma [6,7,8] [Table 1]. In those patients the mid-
dle age was 56,5 and the ratio M:F of 1:3. Tumor size was from 7
to 10 cm, without particular pulmonary localization. Three patients
underwent to lobectomy, our case underwent to pneumonectomy
because of pleural and diaphragmatic infiltration. Only one patient
had recurrency and was treated with RT therapy. The same patient
died after 6 months after diagnosis. Other patients had absence of
disease at follow up after 16, 12 and 4 months rispectively. None
of the patient had metastasies. According to other authors [recen-
sioni trattamento], radical excision with lymphnode dissection is
the best treatment for PPL, and when R0 resection is obtained, no
other treatment is required. Chemotherapy and radiotherapy as ad-
juvant treatment haven’t shown great success even in patients with
residual disease, inoperable cases or metastasies.
clinicsofsurgery.com 5
Volume 7 Issue 8 -2022 Case Report
Table 1
5. Conclusion
Primary polmunar liposarcoma is a extremely rare disease with
potential of local complications and distant metastases. The best
treatment is radical excision. In consideration of the small number
of cases reported in the literature, more studies have to be conduct-
ed in order to have more adequate guidelines.
References
1. Giménez A, Franquet T, Prats R, Estrada P, Villalba J, Bagué S. Un-
usual primary lung tumors: A radiologic‑pathologic overview. Ra-
diographics. 2002; 22: 601‑19.
2. Latienda RI, Itoiz OA: Mixoliposarcoma del pulmon. Arch SOC Ar-
gent Anat Norm Pathol. 1946; 8: 563, 1946.
3. Dey, Treshita. “Rare Case of Primary Pulmonary Pleomorphic Lipo-
sarcoma Treated With Multimodal Therapy.” The Ochsner journal
vol. 2021; 21: 4.
4. Krygier, Primary lung liposarcoma; Achir A. Primary Pulmonary
Liposarcoma… Thorac Cardiov Surg. 2009; 57: 119-124
5. Fletcher CDM, Unni KK (eds). Pathologic and genetics: tumors of
soft tissue and bone World Health Organization Classification of Tu-
mors. IARC Press, Lyon, pp. 2002; 35-46.
6. Chen. Primary intrathoracic liposarcoma: a clinicopathologic study
and prognostic analysis of 23 cases. Journal of Cardiothoracic Sur-
gery. 2014, 9: 119.
7. Longano A, DuGuesclin A, Mitchell C. Primary dedifferentiated li-
posarcoma of the lung with rhabdomyoblastic and chrondroblastic
differentiation. Histopathology. 2015; 67: 923-935.
8. Loddenkemper C, Perez-Canto A, Leschber G. Primary dedifferen-
tiated liposarcoma of the lung. Histopathology. 2005; 46: 710-712.

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Rare Case Of Primary Pulmonary Dedifferentiated Liposarcoma

  • 1. Clinics of Surgery Case Report ISSN: 2638-1451 Volume 7 Rare Case Of Primary Pulmonary Dedifferentiated Liposarcoma Tuoro A¹* , Aversa V² and Cassina EM¹ ¹Thoracic Surgery Unit, “San Gerardo” Hospital, Monza, Italy ²University “Magna Graecia” of Catanzaro, Catanzaro, Italy * Corresponding author: Antonio Tuoro, Thoracic Surgery Unit, “San Gerardo” Hospital, Monza, Italy, E-mail: aversa.vincenzo@hotmail.it Received: 31 Mar 2022 Accepted: 12 Apr 2022 Published: 18 Apr 2022 J Short Name: COS Copyright: ©2022 Tuoro A, This is an open access article distribut- ed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and build upon your work non-commercially. Citation: Tuoro A, Rare Case Of Primary Pulmonary Dedifferentiat- ed Liposarcoma. Clin Surg. V7(8): 1-5 clinicsofsurgery.com 1 1. Abstract Liposarcoma is a relatively common soft tissue sarcoma, but pri- mary pulmonary liposarcomas are extremely rare, expecially for dedifferentiated subtype. We report the case of a 44‑year‑old Af- rican woman, healed from SARS-Covid, who presented with a 8 cm right lung ilary mass and underwent to a right intrapericar- dial pneumonectomy with diaphragmatic and pleural resections. Histological test confirmed diagnosis of dedifferentiated primary pulmonary liposarcoma. The patient recovered well having an un- complicated postoperative course and was discharged after 7 days. On four months follow up the patient was still alive and without evidence of disease. More studies have to be conducted because very few cases are reported in literature. 2. Introduction Primary pulmonary liposarcoma is an extremely rare malignancy with very few cases reported in literature. Intrathoracic liposar- comas are uncommon, and generally located in the mediastinum. Malignant degeneration of a pulmonary lipoma and pleuropulmo- nary asbestosis have been considered as possible pathogenetic fac- tors [1]. Of all subtypes, dedifferentiated is the most uncommon with only four cases descripted in literature. 3. Case Presentation A 44-year-old moroccan woman arrived to our Hospital because of recurring hemoptysis. She underwent about a month before to a Torax-TC that exposed a right lung ilary mass (7,2 cm) treated with antibiotic. An EBUS was performed without bioptic sample because of suspected Echinococcus disease. Once excluded, a transthoracic biopsy was performed, but samples were not clin- ically adeqate because of nectotic tissue. During hospitalization she developed pain associated with hyperpyrexia and increased in- flammation indices, treated with analgesic and antibiotic therapy. A Body-TC [Figure 1, 2] exposed an increment of known mass (8 cm), and body-PET [Figure 2] reported an uneven captation, in particular at the origin of upper right bronchus (SUVmax = 5.84), at middle right bronchus (SUVmax = 6.09) and at middle right bronchus biforcation (SUVmax = 6.03) and no other place worth of notice. So it was decided to perform a right thoracotomy in or- der to reach the mass. Extemporaneous histological test confirmed malignity of the mass, so it was dedided to perform a right pneu- monectomy with diaphragmatic and pleural resections due to infil- tration [Figure 4, 5]. Bronchial stump was covered by intercostal muscle previously isolated. A 32 CH thoracic dreinage was placed. Postoperative period was uneventful, drainage was removed after 2 days and the patient was discharged after 7 days asynthomatic and in good general conditions. On 10 days follow up the patient was still asynthomatic with normal outcomes of pneumonectomy. Histological test confirmed for primary pulmonary liposarcoma dedifferentiated with S100+ and MDM2.
  • 2. clinicsofsurgery.com 2 Volume 7 Issue 8 -2022 Case Report Figure 1: body-TC showing large mass uneven capitation. Figure 2: Thorax-RX.
  • 3. clinicsofsurgery.com 3 Volume 7 Issue 8 -2022 Case Report Figure 3: PET showing. Figure 4
  • 4. clinicsofsurgery.com 4 Volume 7 Issue 8 -2022 Case Report Figure 5 4. Discussion The first case of PPL was reported by Latienda in 1946 [2]. Till then very few cases have been reported in literature. It originates from primitive mesenchymal cell and is common in extremities and retroperitoneum. PPL generally originates in the mediastinum, rarely arises from parenchyma, chest wall or pleura. Possible risk factors associated with development of primary pulmonary lipo- sarcoma are pleuropulmonary asbestosis or malignant transforma- tion of pulmonary lipoma [3]. In our case the patient had contact with asbestos because of the house where she lived, but there was no sign of asbestosis. No particular characteristic symptoms and signs are attributable to PPL but are the same of any other pul- monary tumors, such as cough, sputum production, hemoptysis, dyspnea, chest pain, and loss of weight and appetite. This can lead to further delays diagnosis. Also, because these tumors are slow growing, they often present as a large mass [4]. Diagnosis is based on histological test. The World Health Organization (WHO) clas- sifies liposarcomas into four subtypes [5]: • Atypical well-differentiated, probably benign • Myxoid/round cell, locally aggressive frequently with metas- tasies • Dedifferentiated, very aggressive and higly malignant • Pleomorfic, malignant In literature we found only four cases of dedifferentiated Primary pulmonar liposarcoma [6,7,8] [Table 1]. In those patients the mid- dle age was 56,5 and the ratio M:F of 1:3. Tumor size was from 7 to 10 cm, without particular pulmonary localization. Three patients underwent to lobectomy, our case underwent to pneumonectomy because of pleural and diaphragmatic infiltration. Only one patient had recurrency and was treated with RT therapy. The same patient died after 6 months after diagnosis. Other patients had absence of disease at follow up after 16, 12 and 4 months rispectively. None of the patient had metastasies. According to other authors [recen- sioni trattamento], radical excision with lymphnode dissection is the best treatment for PPL, and when R0 resection is obtained, no other treatment is required. Chemotherapy and radiotherapy as ad- juvant treatment haven’t shown great success even in patients with residual disease, inoperable cases or metastasies.
  • 5. clinicsofsurgery.com 5 Volume 7 Issue 8 -2022 Case Report Table 1 5. Conclusion Primary polmunar liposarcoma is a extremely rare disease with potential of local complications and distant metastases. The best treatment is radical excision. In consideration of the small number of cases reported in the literature, more studies have to be conduct- ed in order to have more adequate guidelines. References 1. Giménez A, Franquet T, Prats R, Estrada P, Villalba J, Bagué S. Un- usual primary lung tumors: A radiologic‑pathologic overview. Ra- diographics. 2002; 22: 601‑19. 2. Latienda RI, Itoiz OA: Mixoliposarcoma del pulmon. Arch SOC Ar- gent Anat Norm Pathol. 1946; 8: 563, 1946. 3. Dey, Treshita. “Rare Case of Primary Pulmonary Pleomorphic Lipo- sarcoma Treated With Multimodal Therapy.” The Ochsner journal vol. 2021; 21: 4. 4. Krygier, Primary lung liposarcoma; Achir A. Primary Pulmonary Liposarcoma… Thorac Cardiov Surg. 2009; 57: 119-124 5. Fletcher CDM, Unni KK (eds). Pathologic and genetics: tumors of soft tissue and bone World Health Organization Classification of Tu- mors. IARC Press, Lyon, pp. 2002; 35-46. 6. Chen. Primary intrathoracic liposarcoma: a clinicopathologic study and prognostic analysis of 23 cases. Journal of Cardiothoracic Sur- gery. 2014, 9: 119. 7. Longano A, DuGuesclin A, Mitchell C. Primary dedifferentiated li- posarcoma of the lung with rhabdomyoblastic and chrondroblastic differentiation. Histopathology. 2015; 67: 923-935. 8. Loddenkemper C, Perez-Canto A, Leschber G. Primary dedifferen- tiated liposarcoma of the lung. Histopathology. 2005; 46: 710-712.