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Year : 2021  |  Volume : 29  |  Issue : 5  |  Page : 56-60

Managing one of the rarest: Recurrent parachordoma

1 Department of Plastic Surgery, Ankara Research and Training Hospital, Ankara, Turkey
2 Department of Pathology, Ankara Research and Training Hospital, Ankara, Turkey
Date of Submission16-Apr-2020
Date of Acceptance26-Apr-2020
Date of Web Publication17-Mar-2021

Correspondence Address:
Dr. Galip Gencay Ustun
Department of Plastic and Reconstructive Surgery, Hacettepe University, Faculty of Medicine, 06100, Altındağ, Ankara
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjps.tjps_40_20

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Parachordoma is one of the rarest tumors identified, with around fifty cases reported to date. It is reported to have a benign behavior, yet metastatic and fatal cases have been reported. A 63-year-old female patient presented with a subcutaneous mass in the right deltoideal region. After two excisional biopsies with tumor-free surgical margins and 33 cycles of radiotherapy, the case presented with a second recurrence. Due to malignant features in the second histopathological examination, she was treated with excision of the deltoid muscle, and the defect was reconstructed with latissimus dorsi myocutaneous flap. There is no standardized treatment protocol for parachordoma. Yet, increased mitotic activity and atypical mitotic figures arise suspicion for recurrent and malignant behavior. In the presence of these features in histopathological examination, tumor should be considered as low-grade sarcoma and be treated accordingly.

Keywords: Atypical mitotic figures, mitotic activity, parachordoma, radiotherapy, recurrence, regional flap

How to cite this article:
Ustun GG, Kargalioglu F, Caydere M, Kocer U. Managing one of the rarest: Recurrent parachordoma. Turk J Plast Surg 2021;29, Suppl S1:56-60

How to cite this URL:
Ustun GG, Kargalioglu F, Caydere M, Kocer U. Managing one of the rarest: Recurrent parachordoma. Turk J Plast Surg [serial online] 2021 [cited 2023 May 30];29, Suppl S1:56-60. Available from: http://www.turkjplastsurg.org/text.asp?2021/29/5/56/311431

  Introduction Top

Parachordoma is one of the rarest tumors identified, with around fifty cases reported to date.[1] Laskowski was the first pathologist to recognize it as a pathological entity and named this tumor “chordoma periphericum” due to the resemblance of chordoma and occurrence in nonaxial sites in contrary to chordomas.[2],[3] Yet, the article published in 1955 could not reach large audiences because it was not in English.[4] Dabska studied five cases reported by Laskowski and five cases of her own and named this tumor “parachordoma” in 1977.[5] Dabska had the opportunity to make a more detailed histopathological evaluation on more specimens. This ten patient case series is still the largest singular patient group ever presented.[5],[6]

When the case reports and small case series are evaluated together, it is observed that these tumors have a slight male predilection (56.8%), and the average age of cases reported is 34.4 (range: 4–86) years. Lower extremities are the most common location of occurrence (51.2%), followed by upper extremities (26.8%).[6],[7] The most common complaint is painless mass; however, pain also has been reported, especially in advanced cases.[8],[9] Parachordomas consist of epithelioid cells, physaliform cells (clear, pale eosinophilic cytoplasm, as similar to chordomas), and spindle cells with narrow cytoplasmic extensions in a hyaline stroma. Epithelioid cells have large cytoplasm and prominent nucleolus and multinucleation.[10] Cytokeratin staining (CK8/18) in immunohistochemistry is also an important feature.[11]

It was reported as a benign tumor with a recurrence rate of 20% by Dabska.[5] Following studies published in 1990s have also reported benign cases with recurrences due to incomplete resection of lesion.[12],[13],[14] However, with the increasing and widespread information about the tumor, metastatic and fatal cases also began to be reported in the following years.[15],[16] Cases have been published at both ends of the behavior spectrum, but the factors that enable predicting behavior have not been revealed yet. Although rare, it is an important tumor for the plastic surgeon as it involves the limbs frequently and can be fatal.

In this study, it is aimed to discuss treatment options for parachordoma through the report of a case.

  Case Report Top

A 63-year-old female patient presented with a subcutaneous mass in the right deltoideal region. The tumor had a 1-year growth history before the presentation. She had no complaint of pain. Examination revealed a 3 cm × 3 cm subcutaneous mass with limited mobilization. There were no skin findings such as ulceration or pigmentation. Motor and sensory examination was normal, and Tinel sign was negative. History reveals that she has hypertension for 10 years and diabetes mellitus for 6 years. Her right leg was amputated transtibially 25 years ago due to a traffic accident. The case was using a cane with her right hand to walk. An informed consent was obtained from the case at the beginning of workup and treatment.

Fixed nature of the tumor made us doubt a potential malignancy, so an ultrasonography (US) was made. In the US, a 55 mm × 33 mm heterogeneous hypoechoic nodular solid lesion which has an indistinguishable border from the muscle was observed in the deltoideal region. Initial diagnosis of radiology was degenerated lipoma. The tumor was excised under local anesthesia after US examination. Yet, during excision, the transparent/creamy appearance of the tumor without a proper capsule strongly suggested that the diagnosis may not be degenerated lipoma [Figure 1].
Figure 1: Intraoperative view of tumor

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In the pathological examination, the dirty cream-colored tumor had fibrous capsule and heterogeneous appearance. Immunohistochemically, positive staining with PanCytoKeratin (PanCK), epithelial membrane antigen (EMA), vimentin, S-100, CK8/18, type 4 collagen, and CD57 was observed. Furthermore, positive staining with actin, calponin, CD31, and CD34 in vascular structures was observed. Negative staining was obtained with Neuron Specific Enolase (NSE), desmin, HHV8, myogenin, Glial Fibrillary Acidic Protein (GFAP), CD68, and CK14. The Ki-67 proliferation index is about 2%–3%. According to histopathological examination, the diagnosis was parachordoma. Borders were tumor free [Figure 2].
Figure 2: (a and b) Tumor consisting of trabecular and cystic structures anastomosed with each other, located in the myxoid stroma, which form lobular structures separated by FB that show infiltration into the subcutaneous adipose tissue and MT (H and E, ×40, ×100). (c and d) The cells that make up the lesion are pleomorphic vesiculonucleus, large cytoplasmic EC with prominent nucleolus, vacuolar cytoplasmic PC, and SC with cytoplasms with narrow extensions (H and E, ×100, ×200). FB: Fibrous bands, MT: Muscle tissue, EC: Epithelioid cells, PC: Physaliform cells, SC: Spindle cells

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Six months after the first excision, the case presented with a recurrence. Two months before the second presentation, she first recognized a swelling. Our examination revealed a subcutaneous mass in the same area [Figure 3]. The characteristics and ultrasonographic features were similar to the first tumor, so a decision for re-excision was made. The lesion was excised with 1 cm borders [Figure 4]. Because of the case is in need of using a cane, the remaining deltoid muscle was salvaged [Figure 5]. The excised mass was 9 cm × 6 cm × 5.5 cm in size.
Figure 3: (a) Positive staining is obser ved with PanCK antibody (immunohistochemistry, ×100). (b) Ki-67 proliferation index is approximately 10% (immunohistochemistry, ×200)

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Figure 4: Preoperative view after the first recurrence

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Figure 5: Intraoperative view of incision planning during the first re-excision

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In the pathological examination, microscopic appearance and immunohistochemical features were same with the first pathological examination. Furthermore, areas of increased mitotic activity and atypical mitoses were observed in the recurrent tumor [Figure 6]. Surgical margins were tumor negative, and no complications were observed in the postoperative follow-up period.
Figure 6: Intraoperative view of the deltoid muscle after tumor re-excision

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After re-excision of the mass, the case was directed to medical oncology and radiation oncology departments due to recurrent and malignant features in pathological examination. While chemotherapy is not recommended due to tumor-free surgical margins, radiotherapy was given by radiation oncologists to prevent recurrence. The case received 33 cycles of radiotherapy (2 Gy/Fx) in a period of 45 days. In the magnetic resonance imaging (MRI) performed 1 month after radiotherapy, a residual recurrent mass approximately 2 cm × 1.5 cm in the posterolateral segments of the deltoid muscle was observed. The mass was not palpable. Because of recurrence for the second time, aggressive features in the second histopathological examination, and relative resistance to radiotherapy, the decision for radical excision was made after consulting with case. After excision, reconstruction with the latissimus dorsi myocutaneous pedicled flap was considered to be appropriate.

The case underwent a radical resection under general anesthesia, and a 13 cm × 10 cm × 5 cm skin-subcutaneous tissue-muscle tissue was excised in one piece, down to the level of the humerus. The defect that occurred after the excision of the mass was reconstructed using a pedicled latissimus dorsi myocutaneous flap [Figure 7]. In the third pathological examination, the mass was white-tanned, irregular, with a slight fusiform fibro-fatty surface with hemorrhage. In the macroscopic view, the tumor was multinodular and composed of areas of central necrosis and hemorrhage. In the microscopic view, there was an anastomosed vesiculonucleus, wide cytoplasmic epithelioid cells, vacuolar cytoplasm cells with narrow nucleus, and narrow extension spindle cytoplasms. Immunohistochemically, positive staining with EMA, vimentin, S100, PanCK, and CK8/18 was observed. Negative staining was obtained with other markers. Margins were free of tumor. No complications were seen in the postoperative period.
Figure 7: Left: Preoperative view of planning for surgical excision and reconstruction with pedicled latissimus dorsi myocutaneous flap. Right: Intraoperative view after flap inset

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After 18 months of follow-up, no recurrence was found in examination or MRI [Figure 8]. The case expressed ability to compensate loss of the deltoid muscle and refused a muscle/tendon transfer for shoulder abduction. A second informed consent was obtained from the case for publishing purposes.
Figure 8: Late postoperative (6 months) result

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  Discussion Top

Etiology of parachordoma is unknown, yet trauma has been suggested as a predisposing factor by Dabska.[5] However, verification of this information is yet to be done. Individual studies reported the absence of trauma in cases with parachordoma.[6],[17] Our case used a cane for walking, and repetitive use of the deltoid muscle may have caused microtearing of muscle fibers and induced trauma. Yet, we cannot reach another parachordoma case with trauma of related region. Tumors presented in visceral areas such as sacral region or retroperitoneum also contradict this hypothesis as these are relatively away from possible trauma.[9],[18]

Parachordoma, although rare, is important for us because it often involves the limbs when it occurs. If it is not encountered by surgeon before, appropriate treatment of the tumor may be compromised. When the published reports are analyzed, there is a lot of controversial data on the behavior of parachordoma, as cases have been published at both ends of the malignancy spectrum.

Despite being described by Laskowski, another Polish Maria Dabska gave the name “parachordoma” to the tumor and is the scientist introduced it to the English literature.[2],[5] After initial description, there is a consensus regarding parachordoma's benign behavior on early publications.[5],[19],[20],[21] During this period, it was stated that the tumor is a slow-growing tumor, and full resection is a cure provider.[7],[22] However, as the information about the tumor became widespread and the frequency of diagnosis increased, multiple cases with malignant behavior also began to be reported. Abe et al. reported a lower extremity parachordoma with neurovascular invasion leading to below-knee amputation. Yet, widespread metastasis to the lung, bone, and skin after the amputation, surgery has developed.[23] The case died after 32 months of follow-up. In this article, they pointed out previous study by Carstens,[24] about an extra-axial “chordoma” that led to loss of a case, may actually be a parachordoma without accurate diagnosis. Abe et al. stated that the possibility of parachordoma to be a low-grade sarcoma could not be excluded and advised to treat accordingly.[23] After this publication, another study from Japan reported a lower extremity parachordoma in a 60-year-old male treated with compartmentectomy of anterior compartment of the leg. After tumor excision, the case experienced hemiplegia, and a brain metastasis was detected in MRI, and the case died 3 months later.[16] De Comas reported a forearm parachordoma with axillary metastasis has led to shoulder disarticulation.[3] Guedes reported a 6-year-old girl with a metastatic parachordoma metastasized to the lungs, refractory to chemotherapy.[15]

In contrary to these malignant cases, there are cases that Dabska has followed nearly 15 years treated as benign tumors without any metastasis or mortality. Hence, is there any explanation for this behavioral discrepancy? Well, local recurrences and metastasis are more frequent in cases with a histologically malignant appearance. In parachordoma cases, metastatic tumors are more cellular, and the cells are more pleomorphic.[25] Necrotic areas and nuclear atypia have been seen in recurrent and metastatic tumors like in this case report.[23]

There is no National Comprehensive Cancer Network (NCCN) or European Society for Medical Oncology (ESMO) guideline to treat parachordoma. Even though parachordoma and chordoma are considered different concepts, there is an NCCN and an ESMO guideline to treat chordomas.[26],[27] In contrast to parachordomas, chordomas have been accepted as aggressively recurring metastasizing and mortal tumors, so interpretation of these guidelines may be helpful to the surgeon. Both of these guidelines suggest surgical excision for primary tumors, radiotherapy for unresectable or recurrent tumors, and chemotherapy/radiotherapy/surgery/supportive care for metastatic disease. They also advise to chordomas with undifferentiated features to be treated as low-grade sarcomas. We think a similar approach is also suitable for parachordomas.

During our practice, sometimes we end up in a crossroads without a clear direction to go. If there are arguments that suggest both ways, take a time-off and discuss these with your patient. Involving patient to every decisional process helps you both medically, mentally, and legally, especially if it is a disease without a standardized treatment protocol. During our first consultation after the first pathology report, the case understood the risks of wide excision and sacrification of the deltoid muscle, the inability to use canes for walking for instance. Specimen did not have any findings indicating malignant behavior, so we made a decision for watchful waiting. Six months after, we faced a recurrence. The same risks were discussed, and a wider excision with preservation of 70% of deltoid muscle was performed. This time, the pathological examination revealed mitotic figures. Guedes reported an ifosfamide/mesna chemotherapy protocol for a metastatic parachordoma case. Moreover, radiotherapy was commonly used in the treatment of chordoma, despite evidence of use in chordomas.[28] Hence, the case was consulted to both medical oncology and radiation oncology departments.[15] Borders were tumor free, so chemotherapy was not necessary at the time. Yet, a radiotherapy protocol to prevent recurrence was performed. Six months after, another recurrence occurred, in spite of radiotherapy. This time, it was easier to decide what to do. The deltoid muscle was sacrificed nearly totally, and reconstruction with latissimus dorsi flap was performed. 18 months after the last operation, there is no recurrence and watchful waiting continues.

Due to its rarity, the literature on parachordoma is growing, and there are no standard treatment protocols. We think that the presence of mitotic figures is important in histopathological examination in predicting malignant behavior. If malignant behavior is predicted, treating as a low-grade sarcoma is appropriate. Radiotherapy does not benefit as in chordomas, primary treatment still appears to be surgery.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]


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