- Email: [email protected]
Intrathoracic Myxoid Spindle Cell Lipoma, A Rare Presentation of a Myxoid Neoplasm
Journal Pre-proof Intrathoracic Myxoid Spindle Cell Lipoma, A Rare Presentation of a Myxoid Neoplasm Steven P. Schulberg, DO, Derek Lim, DO, Syed T. Hoda, MD, Mikhail Vaynblat, MD PII:
S0003-4975(19)31485-7
DOI:
https://doi.org/10.1016/j.athoracsur.2019.08.093
Reference:
ATS 33116
To appear in:
The Annals of Thoracic Surgery
Received Date: 24 July 2019 Revised Date:
21 August 2019
Accepted Date: 23 August 2019
Please cite this article as: Schulberg SP, Lim D, Hoda ST, Vaynblat M, Intrathoracic Myxoid Spindle Cell Lipoma, A Rare Presentation of a Myxoid Neoplasm, The Annals of Thoracic Surgery (2019), doi: https:// doi.org/10.1016/j.athoracsur.2019.08.093. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 by The Society of Thoracic Surgeons
Intrathoracic Myxoid Spindle Cell Lipoma, A Rare Presentation of a Myxoid Neoplasm Running head: Intrathoracic Myxoid Spindle Cell Lipoma
Steven P. Schulberg DO1, Derek Lim DO1, Syed T. Hoda MD2, Mikhail Vaynblat MD1
1) Department of Surgery, NYU Langone Hospital – Brooklyn; Brooklyn, NY 2) Department of Pathology, NYU Langone Hospital; New York, NY
Corresponding Author Steven P Schulberg DO NYU Langone Hospital – Brooklyn Department of Surgery 150 55th Street, Brooklyn, NY 11220 Email: [email protected]
Key Words Myxoid spindle cell lipoma; Incidental lung mass; Chest Wall Tumor
Classifications; Chest; Lung; Lung cancer, diagnosis; Pathology; pleural space
Word Count: 1,108
Abstract: Here we present a case of a 38 year old male with an incidentally found right upper lobe lung mass. The patient underwent thoracoscopic resection of the mass which revealed a myxoid spindle cell lipoma. This is an exceedingly rare location for this tumor biology and here we discuss its pathological features and treatment options.
Myxoid neoplasms are generally benign tumors which can appear in multiple places throughout the body. These tumors have malignant potential as atypical lipomatous tumors (liposarcoma) requiring prompt workup and intervention. Though generally these occur on the upper back and extremities, they have potential for presentation in all areas prompting a high index of suspicion and knowledge of their course during treatment.
This is a 38 year old male with a past medical history of schizophrenia who was evaluated after the finding of an incidental lung mass. The patient initially presented to the emergency department complaining of acute onset, sharp chest pain. The patient had no additional significant past medical history, no past surgical history, no significant family history, and denied a smoking history. On his initial workup, he underwent a chest x-ray (Figure 1) which showed a lobulated pleural mass at the right apex, approximately 3.3 x 7.6 cm. CT chest was performed which revealed a well circumscribed lobulated mass at the right lateral lung apex 5.5 x 4.4 x 3.7 with no evidence of invasion into the chest wall or ribs (Figure 2). The patient was referred for CT guided biopsy of the lesion which revealed a low grade myxoid neoplasm. The patient was subsequently referred to thoracic surgery for definitive management. His additional preoperative workup included PET CT scan (Figure 2) which characterized the lesion as a minimally FDG avid heterogeneously intense pleural-based mass at the right lung apex with a standard uptake value of 2.1. Additional reactive neck and groin lymph nodes were imaged with no gross metastatic disease. Following this workup, the patient was taken for video assisted thoracoscopy and resection of the mass. A densely adherent mass was noted in the right lung apex which was resected without untoward event. The remainder of the hospital course was uneventful and the patient was discharged home on post operative day 2. Final
pathology revealed a low-grade myxoid/adipose tumor, likely myxoid spindle cell lipoma. The mass was histologically described as a hypocellular myxoid spindle cell mass with abundant adipose tissue with mild atypia and no high-grade atypia observed. CD34 staining was strongly present in the myxoid spindle cell component however the mass was negative for MDM2 amplification making the diagnosis of liposarcoma less likely. The patient has been seen for follow up and is noted to be recovering well, he is currently being observed with serial imaging to monitor for recurrence.
Comment The diagnosis of a myxoid spindle cell lipoma within the chest is exceedingly rare. The majority of these masses appear in their normal distribution of the upper extremity, upper back, and head and neck of men.1,2,3 Despite this, a large number of these tumors are found in atypical locations. In a review of the literature, there have been numerous locations for these tumors to appear. They can appear on the trunk and extremities outside of their typical distribution; one review showed the next most common distribution was on the leg and buttock.3 Furthermore, these tumors have been described in the oropharynx and head and neck region. These are typically treated in the same fashion and represent a diversion from the normal distribution. Upon our review of the literature, there was one case of tumor located in the mediastinum but no other reports of any similar tumor found in the chest cavity.4 Histologically, these benign tumors generally appear as mature adipocytes with ropy collagen fibers (Figure 3). A myxoid stroma may be present in these specimens; as was seen in this case. The vast majority of these lesions stain positive for CD34. MDM-2 immunohistochemistry can be used to stain single cells whose positivity may indicate the presence of an atypical lipomatous tumor (also known as well-differentiated liposarcoma). In our case the MDM-2
immunohistochemistry was negative but due to the unusual location additional confirmatory testing using MDM-2 FISH (fluorescent in-situ hybridization) was performed which was also negative for ampflification. With consideration to the morphology, the negative MDM-2 testing, and CD34 positivity the diagnosis of Myxoid Spindle Cell Lipoma was made. Once the pathologic diagnosis has been made, treatment is excision of the tumor. Outcomes are generally positive for these patients and can either be monitored clinically or radiographically for recurrences. Due to their overall rare nature and generally indolent clinical course, no distinct guidelines exist regarding follow up. These patients must be surveilled for chance of recurrence and subsequently treated when present. Although these tumors most commonly occur on the neck, back, and upper extremities, it is important to be mindful of the possibility of atypical location throughout the body.
Figure Legends:
Figure 1: Chest Xray showing right apical pleural mass, approximately 3.3 x 7.6 cm.
Figure 2: (A) Chest CT showing a well circumscribed lobulated mass at the right lateral lung apex 5.5 x 4.4 x 3.7; (B) PET CT showing minimally FDG avid heterogeneously intense pleuralbased mass at the right lung apex with a standard uptake value of 2.1.
Figure 3: (a) Low power, H&E staining; Morphology shows a hypocellular myxoid and spindle cell lesion with intermittent fat present; (b) High power, H&E staining, the myxoid spindle cell stroma shows no significant atypia, the fat shows no significant atypia and appears mature, with noted areas of fibrous collagenized tissue; (c) Medium power, 20x, CD34 staining, vascular structures are highlighted by CD34 as well as spindled stromal cells in dark brown.
References: 1) Enzinger FM, Harvey DA. Spindle cell lipoma. Cancer. 1975;36(5):1852-1859. 2) Billings SD, Goldblum JR. 13 - soft tissue tumors and tumor-like reactions. In: Busam KJ, ed. Dermatopathology. Philadelphia: W.B. Saunders; 2010:499-564 3) Din NU, Zhang P, Sukov WR, Sattler CA, Jenkins SM, Doyle LA, Folpe AL, Fritchie KJ. Spindle Cell Lipomas Arising at Atypical Locations, Am J Clin Pathol. 2016; 146(4):487– 495. 4) La Mantia E, Franco R, Rocco R, Rocco G. Spindle cell lipoma: a rare tumor of the mediastinum. J Thorac Dis. 2013;5(4):E152–E154. 5) Bahadır, B. , Behzatoğlu, K. , Hacıhasanoğlu, E. , Koca, S. B., Sığırcı, B. B. and Tokat, F. (2018), Atypical spindle cell/pleomorphic lipomatous tumor: A clinicopathologic, immunohistochemical, and molecular study of 20 cases. Pathol Int, 68: 550-556. 6) Marino-Enriquez A, Nascimento AF, Ligon AH, Liang C, Fletcher CDM. Atypical spindle cell lipomatous tumor: Clinicopathologic characterization of 232 cases demonstrating a morphologic spectrum. Am J Surg Pathol. 2017;41(2)
S0003-4975(19)31485-7
DOI:
https://doi.org/10.1016/j.athoracsur.2019.08.093
Reference:
ATS 33116
To appear in:
The Annals of Thoracic Surgery
Received Date: 24 July 2019 Revised Date:
21 August 2019
Accepted Date: 23 August 2019
Please cite this article as: Schulberg SP, Lim D, Hoda ST, Vaynblat M, Intrathoracic Myxoid Spindle Cell Lipoma, A Rare Presentation of a Myxoid Neoplasm, The Annals of Thoracic Surgery (2019), doi: https:// doi.org/10.1016/j.athoracsur.2019.08.093. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 by The Society of Thoracic Surgeons
Intrathoracic Myxoid Spindle Cell Lipoma, A Rare Presentation of a Myxoid Neoplasm Running head: Intrathoracic Myxoid Spindle Cell Lipoma
Steven P. Schulberg DO1, Derek Lim DO1, Syed T. Hoda MD2, Mikhail Vaynblat MD1
1) Department of Surgery, NYU Langone Hospital – Brooklyn; Brooklyn, NY 2) Department of Pathology, NYU Langone Hospital; New York, NY
Corresponding Author Steven P Schulberg DO NYU Langone Hospital – Brooklyn Department of Surgery 150 55th Street, Brooklyn, NY 11220 Email: [email protected]
Key Words Myxoid spindle cell lipoma; Incidental lung mass; Chest Wall Tumor
Classifications; Chest; Lung; Lung cancer, diagnosis; Pathology; pleural space
Word Count: 1,108
Abstract: Here we present a case of a 38 year old male with an incidentally found right upper lobe lung mass. The patient underwent thoracoscopic resection of the mass which revealed a myxoid spindle cell lipoma. This is an exceedingly rare location for this tumor biology and here we discuss its pathological features and treatment options.
Myxoid neoplasms are generally benign tumors which can appear in multiple places throughout the body. These tumors have malignant potential as atypical lipomatous tumors (liposarcoma) requiring prompt workup and intervention. Though generally these occur on the upper back and extremities, they have potential for presentation in all areas prompting a high index of suspicion and knowledge of their course during treatment.
This is a 38 year old male with a past medical history of schizophrenia who was evaluated after the finding of an incidental lung mass. The patient initially presented to the emergency department complaining of acute onset, sharp chest pain. The patient had no additional significant past medical history, no past surgical history, no significant family history, and denied a smoking history. On his initial workup, he underwent a chest x-ray (Figure 1) which showed a lobulated pleural mass at the right apex, approximately 3.3 x 7.6 cm. CT chest was performed which revealed a well circumscribed lobulated mass at the right lateral lung apex 5.5 x 4.4 x 3.7 with no evidence of invasion into the chest wall or ribs (Figure 2). The patient was referred for CT guided biopsy of the lesion which revealed a low grade myxoid neoplasm. The patient was subsequently referred to thoracic surgery for definitive management. His additional preoperative workup included PET CT scan (Figure 2) which characterized the lesion as a minimally FDG avid heterogeneously intense pleural-based mass at the right lung apex with a standard uptake value of 2.1. Additional reactive neck and groin lymph nodes were imaged with no gross metastatic disease. Following this workup, the patient was taken for video assisted thoracoscopy and resection of the mass. A densely adherent mass was noted in the right lung apex which was resected without untoward event. The remainder of the hospital course was uneventful and the patient was discharged home on post operative day 2. Final
pathology revealed a low-grade myxoid/adipose tumor, likely myxoid spindle cell lipoma. The mass was histologically described as a hypocellular myxoid spindle cell mass with abundant adipose tissue with mild atypia and no high-grade atypia observed. CD34 staining was strongly present in the myxoid spindle cell component however the mass was negative for MDM2 amplification making the diagnosis of liposarcoma less likely. The patient has been seen for follow up and is noted to be recovering well, he is currently being observed with serial imaging to monitor for recurrence.
Comment The diagnosis of a myxoid spindle cell lipoma within the chest is exceedingly rare. The majority of these masses appear in their normal distribution of the upper extremity, upper back, and head and neck of men.1,2,3 Despite this, a large number of these tumors are found in atypical locations. In a review of the literature, there have been numerous locations for these tumors to appear. They can appear on the trunk and extremities outside of their typical distribution; one review showed the next most common distribution was on the leg and buttock.3 Furthermore, these tumors have been described in the oropharynx and head and neck region. These are typically treated in the same fashion and represent a diversion from the normal distribution. Upon our review of the literature, there was one case of tumor located in the mediastinum but no other reports of any similar tumor found in the chest cavity.4 Histologically, these benign tumors generally appear as mature adipocytes with ropy collagen fibers (Figure 3). A myxoid stroma may be present in these specimens; as was seen in this case. The vast majority of these lesions stain positive for CD34. MDM-2 immunohistochemistry can be used to stain single cells whose positivity may indicate the presence of an atypical lipomatous tumor (also known as well-differentiated liposarcoma). In our case the MDM-2
immunohistochemistry was negative but due to the unusual location additional confirmatory testing using MDM-2 FISH (fluorescent in-situ hybridization) was performed which was also negative for ampflification. With consideration to the morphology, the negative MDM-2 testing, and CD34 positivity the diagnosis of Myxoid Spindle Cell Lipoma was made. Once the pathologic diagnosis has been made, treatment is excision of the tumor. Outcomes are generally positive for these patients and can either be monitored clinically or radiographically for recurrences. Due to their overall rare nature and generally indolent clinical course, no distinct guidelines exist regarding follow up. These patients must be surveilled for chance of recurrence and subsequently treated when present. Although these tumors most commonly occur on the neck, back, and upper extremities, it is important to be mindful of the possibility of atypical location throughout the body.
Figure Legends:
Figure 1: Chest Xray showing right apical pleural mass, approximately 3.3 x 7.6 cm.
Figure 2: (A) Chest CT showing a well circumscribed lobulated mass at the right lateral lung apex 5.5 x 4.4 x 3.7; (B) PET CT showing minimally FDG avid heterogeneously intense pleuralbased mass at the right lung apex with a standard uptake value of 2.1.
Figure 3: (a) Low power, H&E staining; Morphology shows a hypocellular myxoid and spindle cell lesion with intermittent fat present; (b) High power, H&E staining, the myxoid spindle cell stroma shows no significant atypia, the fat shows no significant atypia and appears mature, with noted areas of fibrous collagenized tissue; (c) Medium power, 20x, CD34 staining, vascular structures are highlighted by CD34 as well as spindled stromal cells in dark brown.
References: 1) Enzinger FM, Harvey DA. Spindle cell lipoma. Cancer. 1975;36(5):1852-1859. 2) Billings SD, Goldblum JR. 13 - soft tissue tumors and tumor-like reactions. In: Busam KJ, ed. Dermatopathology. Philadelphia: W.B. Saunders; 2010:499-564 3) Din NU, Zhang P, Sukov WR, Sattler CA, Jenkins SM, Doyle LA, Folpe AL, Fritchie KJ. Spindle Cell Lipomas Arising at Atypical Locations, Am J Clin Pathol. 2016; 146(4):487– 495. 4) La Mantia E, Franco R, Rocco R, Rocco G. Spindle cell lipoma: a rare tumor of the mediastinum. J Thorac Dis. 2013;5(4):E152–E154. 5) Bahadır, B. , Behzatoğlu, K. , Hacıhasanoğlu, E. , Koca, S. B., Sığırcı, B. B. and Tokat, F. (2018), Atypical spindle cell/pleomorphic lipomatous tumor: A clinicopathologic, immunohistochemical, and molecular study of 20 cases. Pathol Int, 68: 550-556. 6) Marino-Enriquez A, Nascimento AF, Ligon AH, Liang C, Fletcher CDM. Atypical spindle cell lipomatous tumor: Clinicopathologic characterization of 232 cases demonstrating a morphologic spectrum. Am J Surg Pathol. 2017;41(2)