A Rare Case of Trichilemmal Carcinoma of the Scalp.

Trichilemmal carcinoma (TC) is a rare, low-grade, malignant adnexal tumor. It is usually less than 3 cm long and arises from the external root sheath of the hair follicle, most commonly in sun-exposed areas of the body. The treatment of choice is wide local excision with tumor-free margins. We present an 88-year-old male patient who presented with an incidental large, dry, fumigating mass on his scalp for a one-year duration requiring surgical excision. The mass, initially thought to be a benign sebaceous cyst, was a 12-cm trichilemmal carcinoma diagnosed based on the histopathologic features of the mass. The specimen was composed of keratinaceous material and necrotic debris. The viable tumor was consistent with atypical squamous proliferation. The mass was fully excised down to the scalp on the first encounter, leaving no further tissue to excise. The patient's scalp site remained clean and without bleeding or recurrence. Currently, there is an increasing incidence of trichilemmal carcinoma. The pathophysiology of this disease is still unclear. The radiation from the sun is one of the factors that causes the growth of the lesions due to its location and distribution. Trichilemmal cysts can also transform into malignant trichilemmal carcinomas due to the p53 deletion. TC has a non-aggressive course despite its aggressive histology. The prognosis is generally good as it has low metastatic potential, like cutaneous squamous cell carcinoma. However, TC with metastasis has a poor prognosis, and there is no consensus yet on treatment. For non-metastatic TC, simple surgical excision with adequate (0.5-1 cm) margins is an effective treatment. Different studies use different margins, and there is no consensus on the measurement for margin excision. Regular follow-up is recommended, but further studies regarding follow-up schedules are needed. Furthermore, despite the common use of chemotherapy in cases of malignant TC, only a limited number of studies have explored this treatment approach. Given the increasing incidence of the disease, we highly recommend more research to address this knowledge gap.

[High-grade Fetal Adenocarcinoma of the Lung with Scalp Metastasis: A Case Report].

Fetal adenocarcinoma of the lung (FLAC) is a rare tumor. Due to its different clinicopathological features, biological behavior and clinical outcome, FLAC is classified into low-grade FLAC (L-FLAC) and high-grade FLAC (H-FLAC). Most patients with H-FLAC are middle-aged heavy smokers. Here, we describe an extremely rare case of a young male patient who denies smoking and initially presents with a mass on the top of the head and is eventually diagnosed with H-FLAC. The aim of this article is to improve the understanding and awareness of FLAC, and increase the attention to the disease, so as to prevent the underdiagnosis and misdiagnosis of the disease, strengthen early identification and accurate diagnosis, and promote subsequent effective treatment and improve prognosis.
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Massive Pilomatrixoma of the Scalp: A Case Report.

Pilomatrixoma, also called epithelioma of Malherbe, is a benign neoplasm derived from hair follicle matrix cells. It usually presents as a solitary mass in the head and neck region and is more frequent in children and young adults, females, and the Caucasian population. Lesions equal to or greater than 5 cm are categorized as giant pilomatrixomas. We present a case of a 75-year-old female, with no known medical history, who was brought to the emergency department (ED) after falling on the street. She had a giant soft head tissue tumor, severe anemia due to intralesional chronic small hemorrhages and folates and cobalamin deficiencies, and delirant speech. The anatomopathological result of the biopsy of the tumor revealed to be a pilomatrixoma. The patient was then referred to plastic surgery, with complete excision of the tumor. After surgery, she was transferred to the psychiatric team, who assumed the delirant speech to be in the context of schizophrenia. She was discharged four months after admission.

Malignant primary tumors of scalp with cranial extension: multidisciplinary surgical strategies and outcomes.

OBJECTIVE: Malignant cancers arising in the scalp may exhibit calvarial invasion, dural extension, and rarely cerebral involvement. Typically, such lesions require a multidisciplinary approach involving both neurosurgery and plastic surgery for optimal resection and reconstruction. The authors present a retrospective analysis of patients with scalp malignancies who underwent resection and reconstruction. METHODS: Patients presenting with scalp malignancies (1993-2021, n = 84) who required neurosurgical assistance for tumor resection were prospectively entered into a database. These data were retrospectively reviewed for this case series. The extent of neurosurgical resection was classified into four levels of involvement: scalp (level I), calvarial (level II), dural (level III), or intraparenchymal (level IV). Complications and evidence of local, locoregional, or regional recurrence were documented. RESULTS: Patients underwent level I (n = 2), level II (n = 61), level III (n = 13), and level IV (n = 8) resections. Pathologies consisted of primarily squamous cell carcinoma (n = 50, 59.5%), basal cell carcinoma (n = 11, 13.1%), and melanoma (n = 9, 10.7%), with infrequent lesions including sarcoma, atypical fibroxanthoma, and malignant fibrous histiocytoma. For cases requiring a cranioplasty, 92.2% were done using titanium mesh and 7.8% with methylmethacrylate. At a mean follow-up of 35.5 ± 45.9 months, the overall survival was 48.8% (n = 41) and recurrence-free survival was 31.0% (n = 43). Scalp-based reconstruction involving plastic surgery was performed in 75 (89.3%) patients. The most commonly used free flap was a latissimus dorsi muscle flap (n = 46, 61.3%). One or more postoperative complications occurred in 21.4% of all patients, the most common being wound dehiscence or delayed wound healing in 13% (n = 11). CONCLUSIONS: A multidisciplinary approach with aggressive neurosurgical resection is associated with good outcomes in patients with primary malignant scalp tumors, despite invasive disease on presentation. This analysis suggests that aggressive resection (level II and higher) is effective at reducing locoregional recurrence and is not associated with a higher risk of complications relative to resection without craniectomy. As most patients require scalp reconstruction to close the postresection defect, usually with vascularized free tissue transfer, involving a plastic surgeon in the surgical planning and execution is essential.

Scalp Tumor and Hydroureteronephrosis in Patients with Nephronophthisis and Homozygous NPHP1 Deletion.

Homozygous deletion of NPHP1 can lead to isolated nephronophthisis (NPHP) and syndromic disorders. However, the phenotype of scalp tumor and hydroureteronephrosis in NPHP patients with homozygous deletion of NPHP1 has not been reported. Clinical data, laboratory results, and genetic testing of 4 NPHP patients were collected. Examination of their eyes, heart, and urinary tract and of their hepatobiliary, skeletal, and central nervous systems was evaluated. Isolated NPHP was observed in 1 case, and syndromic disorders were observed in the other 3 patients. Their syndromic disorders showed NPHP combined with central nervous system defects, eye involvement, scalp tumor, arachnoid cyst, or hydroureteronephrosis. Large homozygous deletions covering the whole NPHP1 gene locus were identified in all 4 patients. We report a novel phenotype of scalp tumor and hydroureteronephrosis in NPHP patients with homozygous deletion of NPHP1, paving an avenue for further research on NPHP1-associated deformity in the skin and the urinary system.

A Rare Case of Diffuse Large B-Cell Lymphoma Found in the Subcutaneous Scalp.

Subcutaneous scalp manifestations of diffuse large b-cell lymphoma are uncommon and can be an easily overlooked diagnosis. Today we report a rare case of a 60-year-old male with a previous history of treatment and removal of multiple benign cysts who presented to the office for the removal of a subcutaneous mass on the left occipital scalp. Intraoperatively the mass did not resemble a cyst. Pathology results showed diffuse large b-cell lymphoma, highlighting the importance of thorough management of subcutaneous masses.

Cranial pilomatricoma: a diagnosis to consider.

Pilomatricomas are benign tumors of the hair follicle that occur frequently in the scalp region. They occur most often in children. We describe a case of pilomatricoma in a teenager, referred to neurosurgery for excision. This diagnosis should be considered in the workup of scalp lesions, and this case report should serve to draw attention to this entity.

Meningothelial Hamartoma Overlying a Prior Craniotomy: A Case Report and Review of Literature.

Meningothelial hamartomas are benign lesions of the scalp with a handful of case reports published. Usually thought to be congenital lesions, they have, on occasion, been seen in older adults. In this report, we describe the first ever reported case of a patient diagnosed with a meningothelial hamartoma overlying a prior craniotomy performed two decades prior. We also briefly describe the literature surrounding these rare lesions, as well as their management and differential diagnosis.

Direct intracranial invasion of eccrine spiradenocarcinoma of the scalp: a case report and literature review.

BACKGROUND: Eccrine spiradenocarcinoma (SC), also known as malignant eccrine spiradenoma, is a rare malignant cutaneous adnexal neoplasm arising from long-standing benign eccrine spiradenoma. Malignant skin tumors rarely show direct intracranial invasion. However, once the intracranial structure is infiltrated, curative excision with sufficient margins can become extremely difficult, particularly when the venous sinuses are involved. No effective adjuvant therapies have yet been established. Here, we report an extremely rare case of scalp eccrine SC with direct intracranial invasion, which does not appear to have been reported previously. CASE PRESENTATION: An 81-year-old woman presented with a large swelling on the parietal scalp 12 years after resection of spiradenoma from the same site. The tumor showed intracranial invasion with involvement of the superior sagittal sinus and repeated recurrences after four surgeries with preservation of the sinus. The histopathological diagnosis was eccrine SC. Adjuvant high-precision external beam radiotherapy (EBRT) proved effective after the third surgery, achieving remission of the residual tumor. The patient died 7 years after the first surgery for SC. CONCLUSIONS: Scalp SC with direct intracranial invasion is extremely rare. Radical resection with tumor-free margins is the mainstay of treatment, but the involvement of venous sinuses makes this unfeasible. High-precision EBRT in combination with maximal resection preserving the venous sinuses could be a treatment option for local tumor control.

Adnexal carcinoma of the scalp: aggressive sebaceous differentiation with invasion of the orbital wall and thoracic vertebra.

Tumors of the scalp are characterized by a heterogeneous clinical spectrum with site-specific features. A wide variety of tumors that arise in the scalp include neoplasms, hamartomas, malformations, and both benign and malignant cysts. Most scalp tumors are benign (98-99%) with only an estimated 1-2% of diagnosed cases reported as being malignant. Of these, adnexal carcinoma is reported in less than 1% of cases. Herein, we report a 65-year-old woman who presented with pain and numbness in her hands with past medical history significant only for a chronic posterior head mass that had never received workup. Computerized topography (CT), magnetic resonance imaging (MRI), and local biopsy demonstrated a large, pedunculated malignant occipital mass that had metastasized to the orbital walls and cervical spine. To our knowledge, this is the largest sebaceous carcinoma to be reported occurring in the scalp. The present case emphasizes the need for older patients with benign scalp lesions to be closely monitored with frequent CT scans for signs of malignant transformation. Furthermore, it is important to diagnose malignant scalp tumors early as they tend to metastasize and cause diffuse symptomatology, which may ultimately result in increased patient mortality.

Giant Malignant Peripheral Nerve Sheath Tumor of the Scalp: Case Report and Review of the Literature.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare nervous system tumors that rarely appear on the scalp. About half of the scalp MPNSTs described in the literature have reached giant dimensions at the time of diagnosis. The surgical treatment is the gold standard for this type of tumor. Some authors suggest adjuvant radiotherapy for local tumor control, although there is uncertainty about its advantages and its use is not without risks. CASE DESCRIPTION: We present the case of a 31-year-old man who presented with a large necrotic scalp tumor of the left frontoparietal convexity. magnetic resonance imaging showed a large extra-axial tumor, measuring 17 x 17 x 8 cm, centered on the soft tissues, with skull erosion and signs of dural invasion, although with no intradural component. The tumor was surgically removed and the osteocutaneous defect was reconstructed with a latissimus dorsi muscle free flap. The anatomopathologic diagnosis was MPNST. The patient then underwent adjuvant radiotherapy. After 7 months he developed a progressive right hemiparesis and magnetic resonance imaging showed results compatible with cerebral radiation necrosis. This motor deficit improved with corticotherapy. After 9 months the patient went back to his home country and was subsequently lost to follow-up. CONCLUSIONS: Giant MPNSTs of the scalp are highly aggressive lesions that should primarily be treated in a surgical fashion. Although adjuvant radiotherapy has been used routinely for local tumor control, there is uncertainty about its advantages.

An Unusual Case of Scalp Metastasis from Breast Cancer.

BACKGROUND: The most common sites of breast cancer metastases are the bone, lung, liver, and brain. Scalp involvement in breast cancer metastasis is extraordinarily rare. CASE DESCRIPTION: This study reports a 52-year-old woman who had a history of malignant right breast cancer and underwent a mastectomy. Positron emission tomography/computed tomography revealed a soft tissue nodule measuring 1 × 0.7 cm located subcutaneously on the top left side of the scalp. A scalp mass excision operation was performed with an extended "S"-shaped incision, and the mass was sent for pathology. Immunohistochemistry showed the following results: CK7: +; ER: 2+, 90%; GATA3: +; GCDFP-15: scattered cells+; mammaglobin: -, napsin A: -; and TTF-1: -. These results were consistent with the characteristics of primary right breast cancer, supporting scalp metastasis from breast cancer. CONCLUSIONS: Scalp metastasis from breast cancer is an exceedingly infrequent phenomenon. Close attention should be paid to soft tissue masses in patients with a healthy appearance and in those with a history of malignant cancer. When neurosurgeons operate on the mass, the circumscription and depth of the tumor must be given further attention.

Dermoscopic follow-up of therapeutic response in mantle cell lymphoma with secondary involvement of the scalp.

The scalp is a potential location for both benign and malignant tumors. Lymphoproliferative diseases can involve the skin as a primary or secondary manifestation. Dermoscopy is a noninvasive diagnostic tool for rapid diagnosis, screening, and follow-up of the majority of skin tumors. Mantle cell lymphoma (MCL), a rare type of aggressive systemic lymphoma, usually occurs as a generalized lymphadenopathy, commonly with infiltration of the bone marrow, spleen, gastrointestinal tract, and Waldeyer's ring. In rare cases, it can also involve other structures, such as the lungs, central nervous system, liver, or skin. We report the case of a 74-year-old male patient suffering from MCL since 2015. Complete remission was obtained after R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) treatment. During maintenance therapy with rituximab, a solitary tumor occurred on the scalp. Dermoscopy of the lesion suggested relapse because of the presence of multiple chaotically distributed short linear vessels with multiple red dots within the hair follicles. Histological examination confirmed the diagnosis of MCL. After second-line therapy with rituximab and bendamustine (R-B), the tumor of the scalp completely disappeared and dermoscopy showed no abnormalities.

The design and clinical applications of the scalp O-Z flap / 中华整形外科杂志

Objective@#To investigate the effect of O-Z flap for the reconstruction of round-or elliptical-shaped defects following the scalp tumor removal.@*Methods@#The resection of the scalp tumor resulted in a round or elliptical-shaped defect. Intraoperative frozen section identified pathological diagnosis, while also ensured margin being tumor-free. Based on the size and location of the defect, the mobility of the surrounding scalp and hair distribution, two local flaps were designed in opposite directions on each side of the defect. The two flaps were rotated to close the defect in an O-Z fashion.@*Results@#From April 2016 to November 2017, 6 patients underwent defect reconstruction using the scalp O-Z flap. The etiologies included basal cell carcinoma (n=2), squamous cell carcinoma (n=2), and trichilemoma and epidermoid cyst (n=2). The defect size ranged from 3.0cm×3.5cm to 5.0cm×6.0cm. Necrosis on the distal of the flap had occurred in 1 patient. It was taken approximately 6% of the flap size, and healed after conservative management. Other flaps survived without any complications. After 3 to 12 months follow-up, the appearance of the sclap was satisfactory and tumor recurrence was not noticed.@*Conclusions@#The O-Z flap technique can be considered as an alternative for the medium-sized defect reconstruction of the scalp. The postoperative outcome of scalp was satisfactory, without disrupted hair orientation.

Malignant Solitary Fibrous Tumor of the Scalp.

Solitary fibrous tumors are an uncommon slow growing benign neoplasm originally described as a pleural neoplasm but can also be found in the lung, mediastinum, peritoneum, or any other sites including the head and neck. Malignant solitary fibrous tumors (MSFT) are extremely rare and only few cases have been published in the literature. There have been 19 cases reported of MSFT in the head and neck, but there are no reports of MSFT located within the scalp in the English language literature. We present a case of MSFT arising in the scalp and describe our experience with the clinical presentation, surgical management, and outcome in this pathological condition.