A 49-year-old man was referred to our institution to evaluate a hypogastric subcutaneous mass of recent onset that experienced very fast growth in the last five months.
Contrast-enhanced CT showed a well-defined, hypogastric subcutaneous soft tissue mass, 45 mm in size. The mass was hyperdense with heterogeneous intralesional pattern. MRI demonstrated a well-circumscribed subcutaneous mass that showed an intermediate T1 signal similar to muscle and low signal on DP, T2 and T2FS images showing small spots and lines of higher intensity with perilesional fat edema and skin engorgement above the mass. It has no significant gadolinium uptake, but there was a thin peripheral enhancement and intense enhancement of the skin above the mass (Figure 2). Ultrasound-guided biopsy showed a hyperechogenic mass without significant vascularization on Doppler (Figure 1B). Initial histologic diagnosis was pilomatrixoma. After surgery, histological examination demonstrated nests of proliferating basaloid cells transitioning to shadow cells. Tumour cells showed atypia and mitoses. This pattern with the presence of shadow cells and increased mitotic activity led to the diagnosis of pilomatrix carcinoma (Figure 3).
Background
Pilomatrix carcinomas (PC) are rare malignant variants of pilomatrixomas, first described in 1980 by Lopansri and Mihm [1].
Clinical Perspective
PC affects more frequently middle-aged and older patients and its usual presentation involves the posterior neck [2,3], upper back and pre-auricular areas [3] and rarely in the trunk, extremities, and genital area [4, 5]. They are usually multicoloured, asymptomatic cystic or solid masses from 0.5 to 14.5 cm [2] with a slow-or fast-growing pattern [6]. PC may develop from benign pilomatrixomas [7]. The diagnosis of PC depends on the presence of histopathologic features: basaloid, ghost, and squamous cells exhibiting varying degrees of atypia and pleomorphism, high proliferative index and an infiltrative growth pattern with central necrosis and infiltration into fat or underlying structures [2, 6]. In our case, cells showed pleomorphism, vesicular nucleus, mitoses, and prominent nucleoli, which are signs of malignancy. However, tumour margin was well circumscribed with no infiltrative changes on both radiological and histological examination, like benign pilomatrixomas [8].
Imaging Perspective
Campoy et al [9] described a recurrent PC of soft tissue in the back invading the spinal canal as having a high signal intensity on T2WI and intermediate signal intensity on T1WI [9]. Niwa et al [3] described a PC of the axilla as a diffusely inhomogeneous mass, with areas of low signal intensity in the T2 WI that corresponded to calcifications, and diffusely inhomogeneous signal intensities observed with contrast enhancement that could relate to varying degrees of proliferating tumour. They described high signal intensity areas in unenhanced T1 and T2 WI that represented cystic spaces in area of tumour necrosis. PC show intense FDG uptake on 18F-FDG PET/CT [4, 10]. In our case CT, MRI and US revealed a well-circumscribed lesion, with T1 homogenous hypointensity, and a slight internal reticular hyperintensity on T2 and T2FS, with ring-like enhancement.
Outcome
Local surgery with wide margins ensures good prognosis and a better survival rate [6]. Radiotherapy may be beneficial [5, 11]. Rate of recurrence varies between wide local excision and simple excision at 23% and 83% respectively. Local recurrence is a predictor of tumour metastasis [5, 6] with a risk between 13% and 16 % [11]. Distant metastasis (lungs, brain, bones, liver and pleura) are very rare and they usually occur in locoregional lymph nodes [5, 10, 12].
Take-Home Message / Teaching Points
PC share characteristics with benign pilomatrixoma. Hystologic analysis of the entire tumour is necessary to reach a definite diagnosis.
Pilomatrix carcinoma
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The patient is a 49-year-old male with a large soft tissue mass observed subcutaneously in the lower abdominal area (suprapubic region). Based on the provided CT, ultrasound, and MRI images, the main imaging characteristics include:
Based on the patient’s middle age, recent rapid growth of the lesion, and imaging findings, possible diagnoses include:
Combining the patient’s age, recent rapid enlargement, the imaging findings, and biopsy results indicating malignant features (including cellular atypia and high mitotic activity), the most likely diagnosis is:
Pilomatrix Carcinoma (Malignant Pilomatrixoma).
As this condition is quite rare, further confirmation with complete surgical pathology and immunohistochemistry may be necessary if there is still doubt. Wide local excision is recommended, and adjuvant treatment (such as radiotherapy) should be considered based on the postoperative pathology.
1. Treatment Strategy:
2. Rehabilitation and Exercise Prescription:
Postoperative rehabilitation should consider the extent of resection and wound healing. It is advised to proceed progressively, following the FITT-VP principle (Frequency, Intensity, Time, Type, Volume, Progression, and Pattern).
By following this step-by-step recovery and exercise plan, along with regular follow-up, patients can improve postoperative functional outcomes and reduce the risk of recurrence.
This report is a preliminary analysis based on the current imaging and pathological information, provided for clinical reference only. It cannot replace in-person consultation or professional medical advice. If there are any questions or changes in symptoms, please consult a relevant specialist or visit a hospital for further examination and treatment.
Pilomatrix carcinoma