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|Year : 2014 | Volume
| Issue : 4 | Page : 400-402
Recurrent malignant proliferating trichilemmal tumor with lymph node metastasis in a young woman
SP Dubhashi, SK Jadhav, A Parasnis, CS Patil
Department of General Surgery, Padmashree Dr. DY Patil Medical College, Hospital and Research Center, Dr. DY Patil Vidyapeeth, Pune, Maharashtra, India
|Date of Submission||07-Aug-2013|
|Date of Decision||30-Oct-2013|
|Date of Acceptance||04-Dec-2013|
|Date of Web Publication||5-Nov-2014|
Dr. S P Dubhashi
Department of General Surgery, Padmashree Dr. DY Patil Medical College, Hospital and Research Center, Dr. DY Patil Vidyapeeth, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Malignant proliferating trichilemmal tumor (MPTT) is a rare cutaneous tumor predominantly affecting the scalp, eyelids, neck and face of elderly women. It is a large, solitary, multilobulated lesion that may arise within a pilar cyst. These tumors are largely benign, often cystic, and are characterized by trichilemmal keratinization. However, at times, the tumor has an aggressive clinical course and a propensity for nodal and distant metastases. Wide local excision with a 1 cm margin of normal tissue is the treatment of choice. Adjuvant chemotherapy and radiotherapy have been occasionally used to prevent recurrence in MPTT. This is a case report of a recurrent malignant trichilemmal tumor over scalp in a young female patient with nodal metastasis.
Keywords: Malignant pilar tumor, nodal metastasis, surgery, trichilemmal tumor
|How to cite this article:|
Dubhashi S P, Jadhav S K, Parasnis A, Patil C S. Recurrent malignant proliferating trichilemmal tumor with lymph node metastasis in a young woman. J Postgrad Med 2014;60:400-2
|How to cite this URL:|
Dubhashi S P, Jadhav S K, Parasnis A, Patil C S. Recurrent malignant proliferating trichilemmal tumor with lymph node metastasis in a young woman. J Postgrad Med [serial online] 2014 [cited 2019 Nov 18];60:400-2. Available from: http://www.jpgmonline.com/text.asp?2014/60/4/400/143973
| :: Introduction|| |
Malignant proliferating trichilemmal tumor (MPTT) is a rare tumor predominantly affecting the scalp of elderly women.  It is a large, solitary, multilobulated lesion that may arise within a trichilemmal cyst (pilar cyst). It yields lobulated and variably exophytic masses that occasionally ulcerate.  The tumor sometimes has an aggressive clinical course and a propensity for nodal and distant metastases.  It is also called as pilar tumor, invasive pilomatrixoma, trichoclamydocarcinoma, proliferating epidermal cyst. Due to its rarity, there are no guidelines available for the management of these tumors. The standard treatment has been wide local excision. The role of radiation therapy and chemotherapy is not established. 
| :: Case Report|| |
A 26-year-old woman presented with a swelling on the right parietal region of the scalp since a year, gradually increasing to the size of 6 x 4 cm. There was no pain or erythema or history of trauma or lymadenopathy. The mass was not fixed to the underlying skull bone. The lesion was excised completely [Figure 1]. Histopathology revealed a proliferating pilar tumor with malignant change. The patient was asymptomatic in the follow-up period of one month.
On Day 30, she again noticed an ulceroproliferative fungating lesion at the same site. It rapidly increased in size over a period of three months, reaching a size of 12 x 8 cm with ulceration of the overlying skin. It was irregular in shape, foul smelling, hard in consistency, fixed to underlying structures with surrounding skin induration [Figure 2]. A single, 1 × 0.5 cm, firm, mobile, non-tender lymph node was palpated in the right posterior triangle of the neck. Wedge biopsy of the growth was consistent with recurrent malignant proliferating trichilemmal tumor. Fine Needle Aspiration Cytology of the lymph node was positive for metastatic deposits. The skull X-ray showed large extra-cranial soft tissue swelling at the right fronto-parietal region.
CT- scan showed a large, well-defined, solid mass measuring 8.1 × 4.6 cm, over scalp in the right fronto-parietal region, suggestive of neoplastic etiology, with possibility of recurrence [Figure 3].
USG abdomen-pelvis and chest X-ray were normal.
Wide local excision with 1 cm margin, with the right side modified neck dissection was done under general anesthesia. The lesion was free from the underlying skull and periosteum. Complete surgical excision was achieved. Reconstruction of the defect was done with a skin graft from the left thigh. The histopathological examination report confirmed the diagnosis. Section showed skin, epidermis appears unremarkable. The dermis showed a well circumscribed neoplasm composed of lobules of squamous cells showing abrupt keratinization. The cells had moderately dense eosinophilic cytoplasm, large hyperchromatic nuclei with prominent nucleoli. Marked nuclear atypia, nuclear pleomorphism and mitotic figures were noted. One of the focus showed peripheral palisading of the nuclei. Foci of necrosis and calcification were also noted [Figure 4]. The base showed fibrocollagenous tissue and was free of tumor. Four out of nine lymph nodes showed metastatic deposits.Post-operatively after one month the patient received adjuvant radiotherapy of 4200 cGy units in 21 sessions over a period of one month. At the point of writing this paper, the patient is disease free.
|Figure 4: Proliferating trichilemmal tumor with trichilemmal keratinization|
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| :: Discussion|| |
Pilar tumors were first recognized by Wilson-Jones in 1966.  They arise through increased epithelial proliferation with pilar or sebaceous cysts. Hence, patients frequently give history of a long standing cyst at the same site.  They are solid or partially cystic, commonly encountered on the scalp, but wrist, elbow, buttocks, chest being other sites for occurrence.  The usual clinical presentation of proliferating trichilemmal tumor (PPT) is that of a long standing, subcutaneous, cystic nodule that slowly progresses to a large nodular mass. Often it follows a history of trauma or inflammation; however, it can also occur de novo.  In our case, there was history of trauma. Histologically, PPT is made up of massively proliferating lobules of squamous epithelium showing multiple central areas of trichilemmal keratinization and formation of homogenous keratin cysts. 
The term malignant proliferating trichilemmal tumor was introduced by Saida et al., because of a proliferating trichilemmal tumor that showed infiltrative growth, marked cytologic atypia, high mitotic activity including atypical forms and lymph node metastases. They suggested three stages in the oncological development of a MPPT- adenomatous stage, epitheliomatous stage and the carcinomatous stage.  Malignant change in pilar tumors conforms to two growth patterns: Circumscribed nodular and diffuse spindle cell type. Differential diagnosis includes sebaceous carcinoma, clear cell hidradenocarcinoma, and cutaneous metastasis of renal cell carcinoma. 
Ye et al., proposed three groups of PPTs. Group 1 PPTs, benign lesions that are non-recurrent, displayed well circumscribed margins, modest nuclear atypia and no pathologic mitoses, necrosis or vascular or neural invasion. Group 2 PPTs, low-grade malignant lesions that may recur, exhibit irregular and locally invasive margins and involvement of deep dermis and subcutis. Group 3 PPTs, high-grade malignant tumors with a tendency toward recurrence and lymph node metastasis, invasive pattern of growth, prominent nuclear pleomorphism, atypical mitoses, necrosis and possible vascular and neural invasion. Group 1 may be regarded as benign, Group 2 as locally aggressive and Group 3 as malignant with metastatic potential. 
Metastasis from malignant pilar tumors has only rarely been reported. Our case had a palpable cervical lymph node when she presented for the second time. HPE confirmed that four out of nine lymph nodes were positive for metastatic deposits. Meta-analysis of 185 patients of 8 reported series confirmed the presence of aggressive variant of the malignant pilar tumor, which has tendency to recur and metastasize distantly. All the patients with aggressive tumors in these reported series were never subjected to any type of adjuvant treatment, which led to an unfavorable outcome. ,,
Wide local excision with a 1 cm margin of normal tissue is the treatment of choice for MPTT. Adjuvant chemotherapy and radiotherapy have been used to prevent recurrence in MPTT.  Our patient received adjuvant radiotherapy and has been kept under close follow-up. Considering the aggressive nature of the malignant variant and high rates of locoregional as well as distant failures in reported series, adjuvant radiotherapy is advocated.
| :: References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]