Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS).
Journal: 2019/September - Autopsy & case reports
ISSN: 2236-1960
Abstract:
Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.
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Autops Case Rep 9(3): e2019110

Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS)

INTRODUCTION

Undifferentiated pleomorphic sarcomas (UPS), one of the entities previously included in the effete diagnosis of malignant fibrous histiocytoma (MFH), most commonly occurs in deep soft tissue of extremities (thigh) of elderly patients (average 50-70 years). So-called MFH was in the past thought to be of fibrohistiocytic origin; however, further studies (electron microscopy, immunohistochemical techniques, or molecular studies) failed to demonstrate evidence of “fibrohistiocytic” differentiation. Additionally, other unrelated poorly differentiated sarcoma was included in this entity, compounding the non-specific and confusing nature of this diagnostic term. MFH was declassified by World Health Organization (WHO) in 2012, and is no longer listed as a diagnostic entity. Many tumors previously included as MFH have been reclassified as “undifferentiated pleomorphic sarcoma”. 1

Primary sarcomas in lung are rare (less than 0.5% of all lung cancers)2, and primary pulmonary undifferentiated pleomorphic sarcomas (PPUPS) are one of the least common primary lung sarcomas, with 84 reported cases in the English literature (Table 1). Weiss SW and Enzinger FM first described soft tissue MFH of 200 cases in 1978.3 A year later, Bedrossian et al.4 reported the first case of primary pulmonary MFH (PPMFH) in a 51-year-old man.

Table 1

Helpful ancillary tests to differentiate tumors with sarcomatoid features
TumorImmunohistochemistryMolecular
Sarcomatoid Carcinoma (SC)P63+, P40+, PanKeratin+, TTF1+, epithelial markers (MOC31, BerEP4, BG8, B72.3, monoclonal CEA)+Gains at chromosomes 8q, 7, 1q, 3q, and 19.
KRAS mutation, EGFR mutation
Sarcomatoid MesotheliomaWT1+, CK5/6+, D2-40+, calretinin+Inactivation of CDKN2A at 9p21 on PCR
Angiomatoid fibrous histiocytoma (AFH)Desmin+/-, CD68+/-, EMA+/-EWSR1-CREB1, EWSR1-ATF1, or FUS-ATF1 fusion
Synovial sarcoma (SS)TLE1+, Keratin+, EMA+, S100+/-, CD56+, CD99+, Calretinin+/-t(X;18) involving SS18 (SYT) gene
Epithelioid Sarcoma (ES)Loss INI, EMA+, Keratin+/-, CD34+/-SMARCB1 (INI1) gene alterations on (22q11)
Dedifferentiated liposarcoma (DDLPS)MDM2+, CDK4+, SMA+/-, Desmin+/-Ring and giant marker chromosomes derived from amplification of 12q13–15 (variable amplification of MDM2, SAS, CDK4, HMGA2)
Anaplastic large-cell lymphoma (ALCL)CD45+, CD30+, ALK+/-TCR gene rearrangement, Rearrangement of 2p23 (ALK)
Inflammatory myofibroblastic tumor (IMT)ALK+/-, SMA+/-, Desmin+/-Rearrangement of 2p23 (ALK)
Ewing SarcomaFLI1+, CD99+t(11;22) and other translocations involving EWSR1 gene
MelanomaS100+, SOX10+, MelanA/MART1+, MITF+, Tyrosinase+BRAF, ARID2, BAP1, GNAQ, HRAS, KIT, NF1, NRAS, and PTEN mutations
Malignant peripheral nerve sheath tumors (MPNST)S100+/-, GFAP+/-. CD34+/-Complex
Solitary fibrous tumor (SFT)STAT6+, CD34+, BCL2+NAB2-STAT6 fusion
Leiomyosarcoma (LMS)SMA+, Desmin+, Caldesmon+Complex
Rhabdomyosarcoma (RMS)Desmin+, Myogenin+, MyoD1+Complex
AngiosarcomaVascular markers (CD31, CD34, FLI1, ERG)+, Keratin +/- in epithelioid angiosarcomaMYC (8q24) or FLT4 (VEGFR3) (5q35) amplification, Upregulation of vascular-specific receptor tyrosine kinases (TIE1, KDR, TEK, FLT1)
Kaposi SarcomaHHV8 (LANA)+, vascular markers+, Lymphatic markers (D2-40, LYVE1, Prox1)+KSHV/HHV8 with PCR
Epithelioid hemangioendothelioma (EHE)Vascular markers+, TFE3+/-, Keratin +/-WWTr1-CAMTA1 fusion, YAP1-TFE1 fusion
Alveolar soft part sarcoma (ASPS)TFE3+, Desmin+/-der(17)t(X;17)(p11.2;q25) translocation (ASPSCR1-TFE3 fusion)
Perivascular epithelioid cell tumor (PEComa)SMA+, Desmin+, HMB45+, MITF+, MART1+TSC2 mutations, TFE3 gene fusions

Yousem and Hochholzer5 reported the most extensive series of primary malignant fibrous histiocytomas in the lung in 1987 with 22 cases (patient age range of 18 to 80 years). Previous irradiation is a known pathogenic risk factor for soft tissue UPS. Similarly, few reports are available in the literature regarding patients who develop PPUPS years after radiation therapy for another tumor.67 It is unclear whether de-novo PPUPS and radiation associated undifferentiated pleomorphic sarcomas represent the same entity.

Clinical presentations in pulmonary tumors, as with other types of lung cancers, depends more on the tumor location rather than the histological type. Primary pulmonary sarcomas often present as a large peripheral or hilar well-circumscribed mass, and may present as an endobronchial tumor in 10% of cases.8 The majority of patients present with symptoms of cough, chest pain, hemoptysis, or dyspnea. Radiologic findings can show a solitary mass with or without post-obstructive effects (recurrent pneumonia, bronchiectasis, lobar or segmental atelectasis), and in some cases with extraluminal growth and/or local invasion into adjacent structures.1

Since clinical and radiographic features of these tumors are nonspecific, pathological tissue examination is required to differentiate them from the much more common epithelial tumors of the lung. The clinical course of these tumors is generally rapidly progressive and metastasis is common.4 The majority of patients die within a period of 1 to 72 months. Endobronchial masses showed more favorable prognosis compare to other origins.8

Here we report the only case of PPUPS that was diagnosed at UCLA from 2002 to 2019.

CASE REPORT

A 66-year-old man who presented with right sided chest wall pain, weight loss (7 kg), fatigue, and night sweats, without any significant past medical history, was found to have normocytic anemia and a large right inferior hemithorax mass on chest X-ray, which was inseparable from the right hemidiaphragm (Figure 1).

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Chest X-Ray (A) anteroposterior view; (B) lateral view showing a large right lower lung mass.

Computed tomography (CT) scan showed a large (15 × 13.6 × 6.2 cm), poorly circumscribed, heterogeneous mass within the right base of the lung involving the pleural surface and mildly protruding into the 8 and 9 intercostal spaces, without eroding of the adjacent bones (Figure 2). Positron emission tomography–computed tomography (PET-CT) scan revealed a large right inferior hemithorax mass with intense fluorodeoxyglucose (FDG) uptake (standardized uptake values (SUVs) of 22.3). The mass abutted the right hemidiaphragm and the chest wall with suggestion of extension into the rib interspaces, most prominent at the right 8 rib interspace. A 6.1 cm right superior paramediastinal mass with intense FDG uptake (SUVMax 16.3) was also seen, suspicious for pleural deposit/metastatic disease (Figure 2).

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Imaging study of the thorax. CT scan (A - axial plane, C - coronal plane) shows a 15 × 6 cm poorly circumscribed right lower lung mass protruding into the 8 and 9 intercostal spaces. (B - axial plane and D - coronal plane) PET scan shows a 15 cm right inferior lung mass and a 6 cm right superior paramediastinal mass with high FDG uptake.

A CT guided core biopsy was subsequently performed. Histological examination revealed a poorly differentiated malignant neoplasm with epithelioid morphology (Figure 3). Microscopic examination did not reveal any line of differentiation by morphology or upon application of immunohistochemistry; tumoral cells were negative for TTF-1, epithelial (pankeratin, EMA, CAM5.2), mesothelial (calretinin), melanocytic (S100 protein, SOX10, MART1), vascular (CD34), and myogenic (desmin, caldesmon) markers. Therefore, the favored diagnosis was poorly differentiated malignant epithelioid neoplasm.

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Photomicrograph of the CT-Guided biopsy showed a poorly differentiated malignant epithelioid neoplasm (H&E, 10X).

Subsequently, the patient underwent surgical wedge resection of right lower and middle lobes, along with the adjacent right chest wall. Gross examination showed a well-circumscribed light tan and firm mass with areas of necrosis and hemorrhage, which abutted the bone without any gross or microscopic evidence of invasion. The mass measured 17.5 cm × 6.5 cm × 1.5 cm (Figure 4).

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Gross examination of the lung mass resection.

Representative sections showed a vaguely nodular mass consists of spindle to epithelioid neoplastic cells arranged in sheets and fascicles some in a storiform pattern. Large, bizarre pleomorphic cells with round to oval nuclei, prominent nucleoli, and moderate amounts of cytoplasm were observed (Figure 5A and and5B).5B). Areas of necrosis (Figure 5C) and high mitotic activity, including atypical mitosis (Figure 5D), were identified.

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Photomicrographs of the tumor showing in A – Vaguely nodular high-grade spindle to epithelioid neoplasm with adjacent rib bone (H&E, 3X); B – Neoplastic cells with adjacent normal lung parenchyma (H&E, 14X); C – Undifferentiated pleomorphic sarcoma with areas of necrosis (H&E, 10X); D – Pleomorphic cells with high mitotic activity, atypical mitoses, chronic inflammatory cell infiltrate and focal necrosis (H&E, 12.5X).

All surgical margins were free of malignancy and no lymph node metastasis was identified. As with the biopsy, the neoplastic cells did not show any immunoreactivity to epithelial, mesothelial, or glandular markers; negative staining for pankeratin, CAM5.2, Keratin 5/6, p63, calretinin, WT1, D2-40, TTF1, S100, SOX10, MART1, HMB45, desmin, caldesmon, EMA, CD34, STAT6, C-Kit, DOG1, myogenin, MyoD1, CD21, CD23, CD35, chromogranin, TLE1, BCL2, and CD99 (Figure 6). Since no evidence of extrapulmonary origin was identified and the tumor did not demonstrate any line of differentiation in our extensive work up, the diagnosis of primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS) was rendered.

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Photomicrographs of the tumor. Immunohistochemistry studies shows no line of differentiation (Magnification x10).

Final Pathologic Diagnosis: (i) Undifferentiated pleomorphic sarcoma, high grade, 17.5 cm; (ii) Surgical margins are uninvolved.

The patient was further treated with radiotherapy and multiple cycles of adjuvant chemotherapy (7 days cycle per month with Ifosfamide 1500mg/m2 CIV on days 1-7). Patient tolerated the treatment without any evidence of local recurrence or metastasis up to this date (4 months follow up).

DISCUSSION

Primary lung sarcomas are rare, and represents less than 0.5% of lung malignancies. Cameron9 and Miller and Allen10 reported primary lung sarcomas in 0.15% and 0.3% of lung neoplasms in studies of 6000 and 10134 patients with primary lung malignancies respectively. Among the primary pulmonary sarcomas, undifferentiated pleomorphic sarcoma (UPS) is one of the rarest one with less than 84 reported cases. UPS is defined as a high-grade pleomorphic neoplasm that shows no discernible microscopic evidence of any specific form of differentiation (e.g., lipoblasts, bone formation, epithelial structures) using currently available diagnostic techniques. Fletcher CD in a retrospective study on re-analysis of 159 tumors showed that just 26% of previously diagnosed MFH cases were “true” UPSs, and more than half of cases showed an identifiable line of differentiation.11 The main core of diagnosis of UPS is to exclude other malignant tumors that display similar morphological findings such as malignant melanomas, sarcomatoid carcinomas, anaplastic lymphomas, sarcomatoid mesotheliomas, or other sarcomas (e.g., dedifferentiated liposarcoma and pleomorphic rhabdomyosarcoma, etc.). Therefore, it is a diagnosis of exclusion, which requires careful examination of tissue and generous sampling of the specimen to stablish a correct diagnosis.12 This diagnosis should not be made definitively on a biopsy specimen, because the entire mass is not present in the biopsy materials. Some authors believe that the category of UPS serves primarily as a “wastebasket” for a heterogeneous group of unclassifiable neoplasms with pleomorphic morphology.13

UPS characteristically shows positive staining for histiocytic markers (CD68, α1-antichymotrypsin, vimentin), a reason why such tumors were identified as “malignant fibrous histiocytoma” (MFH) in the past. Staining for TTF-1, S-100 protein, desmin, actin, myoglobin, caldesmon, D2-40, and calretinin is negative. In some cases, keratin staining may be positive, which makes it difficult to differentiate sarcomatoid carcinomas (SCs) from UPS. Stronger cytokeratin immunoreactivity along with more differentiated carcinomatous elements, as well as immunoreactivity to other epithelial markers (such as P63) can be helpful in the diagnosis of SCs. A wider than usual panel of immunohistochemical studies is necessary to rule out other neoplasms that can resemble UPS including other types of sarcomas, sarcomatoid carcinoma, melanoma, or mesothelioma. (Table 1). Special staining, electron microscopy, or microscopy with ultraviolet surface excitations have not shown strong utility in the diagnosis of UPSs.1415.

Non-specific small droplets of neutral fat and PAS-positive, diastase-resistant droplets may be seen especially in the giant cells in this tumor, which probably reflect a degenerative change.14 Electron microscopic features are helpful for evaluation of better-differentiated tumors; for example, high aspect ratio surface microvilli, perinuclear tonofilaments, and hyaluronic acid crystals are seen in more differentiated mesotheliomas. However, when the tumor is poorly differentiated, the ultrastructural findings are rarely helpful.16 The genetic profile of primary pulmonary mesenchymal tumors shows complex and nonspecific cytogenetic aberrations, similar to their soft tissue counterparts. Molecular studies can be helpful to rule out other tumors with similar histological findings (Table1). Molecular mechanisms responsible for primary pulmonary UPS formation and progression are unknown.14

It is also important to know that UPS is a relatively common soft tissue tumor and most cases of UPS found in the lung represent metastasis from an extra-pulmonary origin.6 Therefore, a careful clinical evaluation to exclude a possible extrapulmonary site of origin is necessary before diagnosing primary pulmonary UPS (PPUPS).

We provide a brief review of literature on PPUPS (previously described as PPMFH) in Table 2. 85 cases have been reported in the English literature since 1979, including our case. 51 out of 85 case were male and 35 were female (M:F ratio of 1.45). The patient age ranged from 9 to 85 years with a mean age of 54. The tumor size ranges from 1.7 to 25 cm, with average size of 7.3 cm, and mean survival of 23.73 months. Even though PPUPS has a high local recurrences and mortality rate, long term survival has been reported in some cases, even more than 10 years.5234952

Table 2

Review of literature of primary pulmonary undifferentiated pleomorphic sarcoma/primary pulmonary fibrous histiocytoma PPUPS/PPMFH.
#YearReferenceAgeSexLocationSize (cm)LNTxSurvival (mos)F/U
11979Bedrossian et al.451MLLL/RML3NL14DOD
21979Kern et al.1753MRLL8NL12DOD
31980Chowdhury et al.752FRLL5UC4DOD
41981Paulsen et al.1853FLLL4NL36DOD
51982Mills et al.1960FRLL10NL18AWD
61982Sriumpai et al.2041MRLL9UL18DOD
71983Misra et al.2145MRLL16PX10DOD
81984Larsen et al.2275MRUL2.5NR10NED
91984Lee et al.2362MLLL6NL12NED
101984Lee et al.2354MLUL7NC7DOD
111984Lee et al.2369MRUL8NPn,X8NED
121984Lee et al.2362FLLL5NL,X120NED
131984Lee et al.2367MLUL4NL60NED
141984Lessel and Erbstösser2435FRLL25UNt12DOD
151984Silverman and Coalson2556MLUL8-C3AWD
161985Tanino et al.2675FLLL5+Nt5DOD
171986Venn et al.2732FRML/RULUUL18NED
181986Venn et al.2762MRUL8UL60NED
191986Venn et al.2761FLULUUPn15DWED
201986Venn et al.2762MLULUUX2AWD
211987Hsiu et al.2871FRUL5NL10NED
221987Juettner et al.2968MLLL20NNt12DOD
231987Juettner et al.2958MRLL5.5PL12DOD
241987Ismailer et al.3012FRUL9NL12AWD
251987Yousem and Hochholzer554FRLL1.7NL108NED
261987Yousem and Hochholzer533MRUL3.8NL84NED
271987Yousem and Hochholzer559MRLL5.9NL65NED
281987Yousem and Hochholzer573FLUL8.5PPn36NED
291987Yousem and Hochholzer564MRUL5NL,X16NED
301987Yousem and Hochholzer542FLLL3NL122NED
311987Yousem and Hochholzer557FRUL4NPn1DNED
321987Yousem and Hochholzer580MLUL3NL1DNED
331987Yousem and Hochholzer574MLULUNNt2DOD
341987Yousem and Hochholzer518MRLL10NL1DOD
351987Yousem and Hochholzer546FRUL6PL,X8DOD
361987Yousem and Hochholzer552FRLLUNC9DOD
371987Yousem and Hochholzer552FLUL4PL,C,X72DOD
381987Yousem and Hochholzer574FRUL14PL24DOD
391987Yousem and Hochholzer569FRUL8NX36DOD
401987Yousem and Hochholzer540FLLL4NL,X24DOD
411987Yousem and Hochholzer574MRMLUNX8DOD
421987Yousem and Hochholzer519MLULUNL,C,X14DOD
431987Yousem and Hochholzer563MLLL7NNt14DOD
441987Yousem and Hochholzer536MRLL3NR12DOD
451987Yousem and Hochholzer532MLLL11PPn,C,X3DOD
461988Casey and Peddle3121MRUL3NL96NED
471988Casey and Peddle3146MLLL10NL8NED
481988McDonnell et al.3273FLLL6.5NL3DOD
491988Palmer et al.3362FRLLUNL14DOD
501989White et al.3455MRULUUNt4DOD
511990In et al.3543FRLLUUC,XUU
521990Marchán and Pérez3610FLLL5NLUU
531993Higashiyama et al.3749FRLL6PPnUNED
541995Kamath et al.3856MRLL10UNt3DOD
551996Gómez-Román and Val-Bernal3961MRUL3UR9NED
561996Halyard et al.4051FLLL10NL,X60NED
571996Halyard et al.4077MRML2.2NL36NED
581996Halyard et al.4040MLLL11PR6DOD
591996Halyard et al.4057FLUL7.5UL1DOD
601996Shah et al.419MLUL6UL,C,X36NED
611997Nistal et al.4212FLUL7UC,X5AWD
621997Barbas et al.4337MRML/RLL10NPn6DNED
632000Fujita et al.4465FLLL12UNt6DOD
642000Herrmann et al.4557MRUL13UL12NED
652001Nonaka et al.659MU4.5UUUU
662002Alhadab et al.4656MLUL/LLLUUNt4DOD
672002Etienne-Mastroianni et al.247MUUUL,X3NED
682003Wang et al.4786MLLL15UNt2DOD
692007Maeda et al.4862MLUL4.5PL24DNED
702007Rzyman et al.4958MLUL4NPn121NED
712007Rzyman et al.4961MRUL7.5NL7DNED
722007Rzyman et al.4975MRUL8PPn4DOD
732007Rzyman et al.4961FLUL3NL2DOD
742007Rzyman et al.4954MRUL9PL3DOD
752008Noh et al.5058FRUL5NL,X5NED
762010Maitani et al.5118FLUL2.2UL36NED
772010Tsangaridou et al.5254MLLLUNPn168AWD
782012Jeon and Park5355MLLLUNPn,C,X9NED
792012Thomas and Koshi5447MRULUPC,X2DOD
802013Kim et al.861MREB3NE14AWD
812013Li et al.5580FRUL8UNt1.5DOD
822014Liu et al.5659FLUL15.7NR8NED
832015Patel et al.5786MRML/RLL9.6NL6NED
842017Li et al.5861MRUL8PLUU
852019This study66MRLL15NL,C4NED

AWD = alive with disease; C = chemotherapy; DNED = dead with no evidence of disease; DOD = death of disease; DWED = death of non-related cause with evidence of disease; E = endoscopic resection; F/U = follow up; F = female; L = lobectomy; LLL = left lower lobe; LN = lymph node; LUL = left upper lobe; M = male; N = negative; NED = no evidence of disease; Nt = no treatment; Pn = pneumonectomy; P = positive; R = resection; REB = right endobronchial; RLL = right lower lobe; RML = right middle lobe; RUL = right upper lobe; Tx = treatment; U = unavailable data; X = radiotherapy.

CONCLUSION

Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS) is an exceptionally rare tumor and is essentially a diagnosis of exclusion. The approach should be to first establish the absence of any particular line of differentiation, which requires proper sampling, histological analysis, and immunohistochemistry studies. Other ancillary tests such as molecular studies and electron microscopy could also possibly be helpful. The next step is to exclude any possible extrapulmonary origin by clinical examination or by other means such as PET-CT scan. PPUPS is a highly malignant sarcoma with a poor prognosis, with surgery being the primary treatment in most cases. Postoperative chemotherapy has also been reported to be beneficial in some cases. We report here the only case of PPUPS, which was diagnosed in the past 17 years at UCLA, after an extensive work up. The patient was treated with surgical resection and post-operative chemotherapy. With 4 months follow up, there is no evidence of local recurrence or distant metastasis.

University of California, San Francisco (UCSF), UCSF Medical Center at Mission Bay. San Francisco, CA, United States of America.
University of California, Los Angeles (UCLA), UCLA Santa Monica Medical Center. Santa Monica, CA, United States of America.
Corresponding author.
Contributed by

Author contributions: Qorbani A and Nelson SD performed pathological and clinical analysis of the case, gave a substantial contribution to the study design, data generation and analysis, writing of the manuscript, and have approved the final version.

Conflict of interest: None
Correspondence
Amir Qorbani
University of California - San Francisco (UCSF) - UCSF Medical Center at Mission Bay
M2369, 1825 4th Street, 94158 – San Francisco/CA – United States of America
CEP: 0000-000
Phone: +1 (949) 609-9916
ude.fscu@inabroq.rima
Conflict of interest: None
Received 2019 Mar 27; Accepted 2019 Jul 15.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the article is properly cited.

Abstract

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.

Keywords: Lung, Lung Neoplasms, Sarcoma, Soft Tissue Neoplasms
Abstract

AWD = alive with disease; C = chemotherapy; DNED = dead with no evidence of disease; DOD = death of disease; DWED = death of non-related cause with evidence of disease; E = endoscopic resection; F/U = follow up; F = female; L = lobectomy; LLL = left lower lobe; LN = lymph node; LUL = left upper lobe; M = male; N = negative; NED = no evidence of disease; Nt = no treatment; Pn = pneumonectomy; P = positive; R = resection; REB = right endobronchial; RLL = right lower lobe; RML = right middle lobe; RUL = right upper lobe; Tx = treatment; U = unavailable data; X = radiotherapy.

Footnotes

How to cite: Qorbani A, Nelson SD. Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS). Autops Case Rep [Internet]. 2019 Jul-Sep;9(3):e2019110. https://doi.org/10.4322/acr.2019.110

The institutional patient's consent was retained, and the manuscript is by the Institutional Ethics committee.

Financial support: None

Footnotes

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