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Radiol Case Rep
Radiol Case Rep
Radiology Case Reports
1930-0433
Elsevier

S1930-0433(24)00689-7
10.1016/j.radcr.2024.07.103
Case Report
A rare cause of hemoptysis: Primitive pulmonary synovial sarcoma
Graini Soumya EL MD elgrainisoumya@gmail.com
a⁎
Laasri Khadija MD a
Amalik Sanae PhD a
Bakkari Assaad El MD a
Allioui Soukaina MD a
Essaber Hatim MD a
Abbad Faycal MD b
Jerguigue Hounayda MD a
Latib Rachida PhD a
Omor Youssef PhD a
a Radiology Department, National Institute of Oncology, Mohamed V University Rabat, Rabat, Morocco
b Anatomo-pathology Department, Cheikh Zaid International Teaching Hospital, Rabat, Morocco
⁎ Corresponding author. elgrainisoumya@gmail.com
22 8 2024
11 2024
22 8 2024
19 11 51395143
24 3 2024
16 7 2024
17 7 2024
© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Synovialosarcoma is a mesenchymal tumor with soft tissue predilection, metastasizing to various organs, including the lung. Primary pulmonary involvement is rare and requires histological examination for confirmation. In early stages, the treatment is surgical followed by radiotherapy to avoid recurrence, while there's no consensus in chemotherapy. We present a case of a 54-year-old patient with primary pulmonary synovialosarcoma. The patient underwent successful treatment, achieving tumor control and complete excision. This case discusses diagnostic approaches, prognosis, treatment modalities for primary pulmonary synovialosarcoma, emphasizing the significance of early intervention for favourable outcomes.

Keywords

Primitive pulmonary synovialosarcoma
Hemoptysis
Chest imaging
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pmcIntroduction

Synovialosarcoma (SS) is a mesenchymal tumor that usually develop in soft tissue, especially in periarticular one. However, cases of other localization have been reported, such as the trunk, the abdomen, and the head. Due to the rarity of primary pulmonary synovialosarcoma, the diagnosis should be meticulous, with careful histological examination to avoid mistaking it for a metastatic lesion. The primary treatment for nonmetastatic primary pulmonary synovialosarcoma is complete surgical removal, with adjuvant radiotherapy recommended for tumors larger than 5 cm. While chemotherapy's role is unclear, some studies suggest using ifosfamide for unresectable or metastatic cases to improve survival. We report the case of a primary synovialosarcoma of the lung in a 54-year-old woman.

Case presentation

It's about a 54-year-old woman with no previous medical history, who was suffering from haemoptysis, cough and respiratory distress. The clinical examination wasn't conclusive, leading to a chest X-ray (Fig. 1) showing a “voluminous left supra-hilar opacity with a clear external limit”, completed by a thoracic CT-scan (Fig. 2) revealing an apico-ventral tumor of the upper left lobe, with a pleural and vascular contact especially with the left branch of the pulmonary artery. Then, a scan-guided biopsy was performed, followed by an anatomical-pathological study which came back in favour of a malignant tumor proliferation with a mesenchymal appearance (Figs. 3A and B).Fig. 1 Frontal chest X-ray showing a left upper lobar opacity.

Fig 1

Fig. 2 CT scan of the left upper lobar tumor process with vascular (red arrow) and pleural (blue arrow) contact.

Fig 2

Fig. 3 (A) Micrograph (Gx10) showing a malignant mesenchymal tumor proliferation. It is of high cell density, arranged in intersecting, swirling bundles. It has a high cell density, arranged in intersecting, swirling bundles. (B) Micrograph (Gx40) showing spindle-shaped tumor cells with ovoid nuclei and some moderate atypia.

Fig 3 (

The immunohistochemical complement showed a diffuse membrane expression of EMA antigens, moderate and diffuse cytoplasmic expression of AML antigen and nuclear expression of Ki67 antigen in 15% of tumor cells. Furthermore, there was no expression by the tumor cells of Ck 5/6, Calretinin and S100 antigens. This led to the conclusion of a monophasic synovialosarcoma classified as grade II of the FNCLCC (tumor differentiation 3, mitotic index 1, tumor necrosis 1). The patient underwent a CT-scan in order to evaluate the extension of the tumor, which did not find any metastasis or malignant lesion, thus confirming the primary pulmonary nature of SS.

Given the local extension and the contacts with the pleura and the left branch of the pulmonary artery, the therapeutic attitude consisted of 3 courses of neoadjuvant chemotherapy including uromitexan, ifosfamide and doxorubicin. The evolution post chemotherapy was good with a clear regression of the tumor size making it resectable (Fig. 4). The patient underwent a left upper lobectomy and complete lymph node dissection, with a pathological study reconfirming the diagnosis of primary monophasic pulmonary synovialosarcoma.Fig. 4 CT-Scan performed after the chemotherapy showing the regression of the tumor size and diminution of the pleural and vascular contact (blue and red narrow).

Fig 4

Follow-up CT scans for her disease did not reveal any tumor recurrence or secondary location, after a 3-years observation period (Fig. 5).Fig. 5 CT-Scan performed after the lobectomy showing no sign of recurrence.

Fig 5

Discussion

Synovialosarcoma (SS) is a rare, malignant, and highly aggressive mesenchymal tumor, constituting 2.5%-10% of sarcomas. Its pulmonary occurrence is extremely rare, accounting for <0.1% of lung cancers. Despite its name, SS is unrelated to synovial cells and is believed to stem from pluripotent mesenchymal stem cells. The nomenclature is based on its microscopic resemblance to synovial cells and its frequent location in the paraspinal area [1,2].

In fact, it is located in 80% of cases at the para-articular level and 20% in various anatomical sites, unrelated to the synovial tissue. The most affected sites in this case are, in order of frequency: the trunk (8%), the abdomen and the retroperitoneum (7%), the head, and neck (5%) [3,5]. It most often affects young adults around 40 years of age but can also impact older individuals, with a slight male predominance [4].

Due to the rarity of primary pulmonary synovialosarcoma (PPSS) and the frequent secondary pulmonary locations of SS in other sites, the diagnosis of PPSS requires first ruling out pulmonary metastasis, and correlating the results of clinical, radiological, histological, immune-histochemical, and cytogenetic data [1,5].

The mode of revelation of the PPSS is dominated by respiratory symptoms. This may include cough, haemoptysis, dyspnoea or respiratory discomfort. When the location is peripheral, it is asymptomatic and it is only when it invades adjacent structures such as the pleura and chest wall that the patient may present with chest pain. Secondary locations of PPSS may also prompt consultation and reveal the tumor [1,3,6].

Radiologically, the imaging of PPSS is usually nonspecific, having the same radiological aspect as lung tumor. The radiographic X-ray shows a well-limited opacity, and the CT scan shows a well-circumscribed, heterogeneously enhancing mass, sometimes containing areas of necrosis or calcifications.

It is also advisable to conduct a cerebral and abdominal-pelvic extension assessment to rule out secondary locations and confirm the primary site. Mediastinal lymph node involvement is uncommon. While the efficacy of PET-SCAN in diagnosing SS is not thoroughly studied, it aids in assessing disease extent and identifying potential secondary locations. Bone scintigraphy is recommended only if there is suspicion of bone involvement [1,7,8].

Regarding the histological aspect, there are frequently 2 main subtypes of SS, namely the monophasic, which corresponds to the pure fibrosarcomatous form made up solely of spindle cells, and the biphasic, where both spindle cells and epithelial cells are found. There are also a few rare subtypes such as the poorly differentiated form, containing small, oval-shaped cells characterised by sparse cytoplasm and a dense nucleus; the monophasic epithelial form; the myxoid form and the calcified or ossified form.

The immunohistochemistry analysis is crucial to rule out other diagnosis. Indeed, in the majority of cases, the expression of epithelial markers such as EMA (epithelial membrane antigen) as well as cytokines is found, in 60% of cases CD99 and in 30% of cases S100 protein, it can also express a reactivity towards the calretinin protein.

The use of RT-PCR or FISH techniques also confirms the diagnosis by showing a characteristic translocation t(X,18), which involves the SSX1 or SSX2 genes of the X chromosome (Xp11) [4,[9], [10], [11]].

The prognostic factors for PPSS do not differ from those of the other sarcomatous localisations. In addition to the presence of metastases at the time of diagnosis, there are some predictive factors including age >20 years and female gender. Tumor diameter greater than 5 cm, neurovascular invasion or incomplete surgical resection are also poor prognostic factors. From a histopathological perspective, we observe extensive tumor necrosis, large number of mitotic figures (>10/10 high-powered fields), as well as a high histopronostic grade according to FNLCC (Fédération Nationale des Centres de Lutte Contre le Cancer) [11,6]. It is estimated that the 5-year survival rate is about 60% and the 10-year survival rate is 50% [11].

The treatment of choice for the PPSS in the absence of metastatic location is surgical removal, as long as the tumor remains resectable. Considering the risk of local recurrence, it is imperative that the surgery be as complete and extensive as possible.

Adjuvant radiotherapy is appropriate for tumors >5 cm in diameter, as SS is considered a high-grade tumor, it allows for better control of the tumor site. Unlike soft tissue SS which is chemosensitive, there are no clear data regarding the place of chemotherapy in PPSS. Nevertheless, some studies recommend the use of ifosfamide in certain patients, especially if the tumor is unresectable or metastatic, thus increasing survival [9,5,12].

Conclusion

PPMS is a rare and highly aggressive lung tumor. Symptomatology is dominated by respiratory signs and the radiographic appearance is nonspecific. The 2 most frequent histological aspects are monophasic and biphasic. The combination of surgery and radiation therapy has been shown to be highly successful. The use of neo-adjuvant chemotherapy can also help to improve the surgical management of the patient.

Guarantor of submission

The corresponding author is the guarantor of submission.

Author contributions

All authors contributed to this work. All authors have read and approved the final version of the manuscript.

Patient consent

Written informed consent for publication was obtained from patient.

Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
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References

1 Pandey L. Joseph D. Pasricha R. Gupta M.K. Primary synovial sarcoma of the lung: a rare presentation, diagnostic dilemma and review of literature BMJ Case Rep 13 11 2020 1 4 10.1136/bcr-2020-237678
2 Gupta A. Palkar A. Narwal P. Kataria A. Pulmonary synovial sarcoma Respir. Med. Case Rep 25 September 2018 309 310 10.1016/j.rmcr.2018.10.015 30386721
3 Ammar A. El Hammami S. Sellami Kamoun N. Une localisation primitive inhabituelle du synovialosarcome: le poumon Rev Mal Respir 23 3 2006 285 287 10.1016/s0761-8425(06)71581-9 16788532
4 Dermawan J.K.T. Policarpio-Nicolas M.L.C. Cytological findings of monophasic synovial sarcoma presenting as a lung mass: report of a case and review of the literature Diagn Cytopathol 47 9 2019 948 955 10.1002/dc.24218 31173483
5 Eilber F.C. Dry S.M. Diagnosis and management of synovial sarcoma J Surg Oncol 97 4 2008 314 320 10.1002/jso.20974 18286474
6 Falkenstern-Ge R.F. Martin K. Andreas G. Heike H. Godehard F. German O. Primary pulmonary synovial sarcoma: a rare primary pulmonary tumor Lung 192 1 2014 211 214 10.1007/s00408-013-9521-1 24170216
7 Duran-Mendicuti A. Costello P. Vargas S.O. Primary synovial sarcoma of the chest: radiographic and clinicopathologic correlation J Thorac Imaging 18 2 2003 87 93 10.1097/00005382-200304000-00006 12700482
8 Remy O. Messouna M. Akimana G. Kamdem M. Errihani H. Le synovialosarcome du poumon: à propos d'un cas et revue de la littérature Pan Afr. Med. J. 36 137 2020 1 6 10.11604/pamj.2020.36.137.23034 32550964
9 Mbatchou Ngahane B.H. Baudrand H. Traverse-Glehen A. Freymond N. Guibert B. Pacheco Y. Évaluation des facteurs pronostiques du synovialosarcome thoracique Rev Mal Respir 27 1 2010 93 97 10.1016/j.rmr.2009.11.006 20146960
10 Bhattacharya D. Datta S. Das A. Halder K. Chattopadhyay S. Primary pulmonary synovial sarcoma: a case report and review of literature Int. J. Appl. Basic Med. Res. 6 1 2016 63 10.4103/2229-516x.174019 26958527
11 Thway K. Fisher C. Synovial sarcoma: defining features and diagnostic evolution Ann Diagn Pathol 18 6 2014 369 380 10.1016/j.anndiagpath.2014.09.002 25438927
12 Singhal S. Prajapat D. Sharma R. Talwar D. Primary pulmonary synovial sarcoma: is it worth all the hard work? J. Assoc. Chest Physicians 7 1 2019 29 10.4103/jacp.jacp_15_18
