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Tumor Discovery                                                                MPTT post-chemotherapy














                                           Figure 2. Mapping of the patient’s nodular scalp lesions.

                         A                       B                        C













            Figure  3. Malignant proliferating trichilemmal tumor. (A) A mid-dermal lobulated proliferation with (B) trichilemmal keratinization, (C) marked
            cytonuclear atypia and numerous mitosis.

            aneuploidy, necrosis, and high mitotic activity, especially   tendency for distant metastasis. Our case aligns with the
            atypical mitosis. In addition, vascular and neural   classification of high-grade MPTT. Additional differential
            invasion may also be observed. 2,5,7,18  These histological   diagnoses of MPTT include dermoid cyst, basal cell
            features, as well as the positivity of proliferation markers   carcinoma, keratoacanthoma, invasive Bowen’s disease,
            such  as  Ki67  and  p53,  and  the  presence  of  lymph  node   trichoblastoma, cylindroma, spiradenoma, sebaceous
            or distant metastasis, are essential in distinguishing   carcinoma, clear cell hidradenocarcinoma, and pilomatrix
            MPTT from PTT.  1,2,4,19  Trichilemmal keratinization is   carcinoma. 3,11,14,16
            also an important indicator in differentiating PTTs from   MPTTs are characterized by aggressiveness, with
            squamous cell carcinomas;  it is characterized by abrupt,   estimated rates of local recurrence and lymph node
                                  2
            compact, amorphous keratinization of epithelial cells   metastasis ranging from 3.7 to 6.6% and 1.2 to 2.6%,
            enveloping the cyst wall in the absence of a granular cell   respectively. 10,13,21  The true metastatic rate of MPTT remains
            layer. 1,2,4,9,13  Immunohistochemical studies are also valuable   unknown, yet reports indicate figures as high as 25% for
            in distinguishing MPTT from squamous cell carcinoma.   grade  III lesions. 3,20,21  Metastases have been reported at
            CD34, an important immune determinant indicating   various stages, ranging from initial presentation to as late
            trichilemmal differentiation, is known to be expressed   as 10 years thereafter.  Consequently, a radical approach to
                                                                                6
            in MPTTs but not in squamous cell carcinomas. 1,2,4,19    treatment is warranted for MPTTs.
            Nevertheless, negative staining with CD34 has been
            detected in several case reports of MPTTs. 2,4,17  Based on   Primary treatment typically entails surgical excision
            clinicopathological findings, Ye  et al.  classified MPTTs   with a 1 cm margin of uninvolved tissue. 3,9,11,16  For current
                                          20
            into  three  groups:  Group  I  comprises  benign  lesions   lesions, certain authors advocate for a 2 cm margin. Mohs
            with regular histological contours, modest nuclear   surgery has been proposed as a potentially less invasive and
            atypia, and absence of pathologic mitoses, necrosis, or   more efficient technique. 22,23  However, the literature lacks
            nerve/vessel invasion, with no reported recurrence.   conclusive  evidence  regarding  the  contiguity of  MPTTs.
            Group II encompasses low-grade malignant tumors with   Non-contiguous lesions negate the benefit of Mohs surgery,
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            irregular histological contours and local invasion into   as skip lesions lead to inaccurate clearance margins.  The
            the deep dermis and subcutis, with potential for local   role of adjuvant chemotherapy or radiotherapy remains
            recurrence. Group  III consists of high-grade malignant   debatable, particularly in cases of localized disease, due to
            tumors  exhibiting  invasive  growth  patterns,  remarkable   the exceptional nature of the condition, requiring further
            nuclear atypia, atypical mitoses, geographic necrosis,   evaluation. Certain authors consider it unnecessary,
                                                                                                            3,9
            and  involvement  of  nerves  or  vascular  structures,  with   while others have used adjuvant radiotherapy to the
            a high recurrence rate, lymph node involvement, and a   treated area and neck to prevent recurrence  or to reduce
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            Volume 3 Issue 2 (2024)                         3                                 doi: 10.36922/td.2344
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