INTRODUCTION

Basal cell carcinoma (BCC) is the most common form of skin cancer, originating in the basal cells of the epidermis, typically arising in sun-exposed areas due to chronic UV exposure, particularly in fair-skinned individuals. While BCC is generally indolent and rarely metastasizes, certain variants, such as giant basal cell carcinoma (GBCC), exhibit more aggressive behavior [1]. Defined by a tumor size exceeding 5 cm (T3) [2], GBCCs are rare and can lead to significant morbidity due to local invasion. Their rarity and association with delayed treatment or underlying conditions, such as psychiatric disorders or immunosuppression, underscore the importance of early detection and management [1,3].

CASE REPORT

A 47-year-old woman with a history of stable chronic psychosis presented with a foul-smelling, ulcerated tumor on her right breast, evolving over ten years and progressively increasing in size. Despite her high socioeconomic background, she delayed seeking medical care due to fear until the odor compelled her family to intervene.

Clinical examination revealed a 21 cm ulcer with irregular borders, a purulent surface interspersed with greenish pseudo-membranes, and exogenous deposits. The lesion involved the outer quadrants of the right breast and extended into the sub-axillary region. The surrounding tissue was inflamed and infiltrated, covered by an erythematous plaque with erosions and a whitish coating (Fig. 1). Differential diagnoses included inflammatory breast cancer, non-melanoma skin cancers, and pyoderma gangrenosum.

Figure 1: Clinical examination: A large, well-defined, 21 cm ulceration with irregular borders and a purulent surface interspersed with greenish pseudo-membranes and exogenous deposits, involving the two outer quadrants of the right breast and extending into the sub-axillary region. The surrounding breast tissue was swollen, inflamed, and covered with an erythematous, infiltrated plaque dotted with erosions and a whitish coating extending to the axillary fossa.

Local wound care, along with bacterial and fungal infection treatment, resolved the inflammation and prepared the lesion for clinical and dermoscopic evaluation (Fig. 2). Dermoscopy of raised borders revealed blue-gray blotches with concentric structures (Figs. 3a and 3b), initially thought to be exogenous deposits. Imaging showed reactive axillary adenitis and diffuse breast tissue thickening up to 9 mm with significant mammary gland infiltration but no distant metastasis.

	Figure 2: Clinical examination after local wound care and treatment of superinfections: large bleeding ulceration with irregular, pigmented borders.
	Figure 3: (a) Dermoscopy: pigmented globules, concentric structures. (b) Dermoscopy: blue-grey blotches.

The patient underwent surgical excision of the tumor with 1 cm lateral and deep margins, including the affected subcutaneous fat and part of the pectoral muscle. The nipple-areola complex was amputated, and the intraoperative frozen section confirmed clean margins (Fig. 4a). Hemostasis, dead space closure, and suction drain placement were performed. In a second step, A split-thickness skin graft from the anterior right thigh was applied over the defect (Fig. 4b). However, graft rejection and necrosis occurred due to neglect and inadequate wound care, necessitating directed healing with LED phototherapy and specialized dressings.

Figure 4: (a) Surgical excision of the tumor with 1 cm margins, including subcutaneous fat and a part of the pectoral muscle. Nipple-areola complex amputation. (b) A split-thickness skin graft from the anterior right thigh applied to the defect after hemostasis, dead space closure, and drain placement.

Histopathology showed irregular tumor islands with atypical basaloid cells, diminished peripheral palisading, stromal hyalinization, and low cellular differentiation, consistent with aggressive nodular and infiltrative basal cell carcinoma. The patient is being evaluated by a multidisciplinary team for adjuvant radiotherapy.

DISCUSSION

GBCCs are rare, aggressive BCC variants, defined as lesions exceeding 5 cm in diameter (T3) [2], regardless of histological subtype, local invasion, or metastasis, although each of these features can be present [4]. They often result from delayed care, frequently linked to factors such as psychiatric disorders, immunosuppression, or negligence [1,3]. Unlike typical BCCs predominantly occurring in sun-exposed areas, GBCCs tend to involve the trunk, likely reflecting differences in detection patterns or underlying risk factors.

Due to its rarity, GBCC has been the subject of limited research, with existing literature mainly including case reports and small case series. A notable series from a U.S. tertiary center reviewed 50 GBCC cases over four years, reporting that most developed from previously treated BCCs [5]. In contrast, a study analyzing 43 GBCCs reported 84% as de novo, while only 16% arose from previously treated BCCs [6]. Despite differing origins, neither study reported metastasis, emphasizing the importance of delayed medical attention and high-risk histological subtypes in the development of GBCC. Similarly, a series of 37 GBCC cases also reported no metastases, highlighting tumor behavior variability [7].

While most large series report no metastatic cases, GBCC still has a higher potential for metastasis than conventional BCC. Most GBCCs are locally or distally advanced at the time of presentation, and several case reports describe metastasis. Among these, three cases involved lung metastases [8–10], one case reported metastases to the lungs, liver, mediastinum, and adrenal glands [11], and older reports described lymph node involvement. These rare cases highlight GBCC’s aggressive nature and the need for further research on metastatic progression.

An even more extreme variant, super giant basal cell carcinoma (SGBCC), exceeds 20 cm in size, surpassing GBCC in both scale and complexity. These cases, like ours, highlight the devastating impact of prolonged neglect and delayed treatment, reinforcing the need for timely intervention. Contributing factors to this neglect may include severe psychiatric conditions such as autism or schizophrenia, self-neglect, lack of insurance, inadequate treatment of prior tumors, chronic alcoholism, and medical avoidance. As a result, SGBCCs can lead to significant local invasion, extending into deeper tissues such as the dermis, muscles, and bones.

A recent systematic review of 20 SGBCC cases revealed a higher prevalence in Caucasian males. Most of these tumors were located in areas covered by clothing, typically the trunk, and their growth was often fueled by ongoing patient neglect. The duration of the lesions varied significantly, ranging from 1 to 30 years, with the majority evolving for over a decade. Metastasis was observed in seven patients, while the remaining 11 did not present with metastatic spread [12]. These findings highlight the considerable variability in SGBCC presentation and progression, underscoring the importance of early detection and intervention to mitigate further morbidity and mortality. Even in the absence of metastasis, SGBCCs can result in severe morbidity and death due to complications such as severe anemia and hypoproteinemia caused by continuous blood and exudate loss, as well as serious infections and sepsis.

Our case underscores the destructive potential of SGBCC when neglected. The tumor in this case reached 21 cm in diameter, with ulceration, superinfection, and infiltration of surrounding structures. The clinical presentation was particularly challenging, mimicking inflammatory breast cancer and pyoderma gangrenosum, which highlights the diagnostic difficulties posed by such advanced cases. This case further exemplifies the devastating consequences of delayed treatment and emphasizes the critical need for early detection to prevent the extensive morbidity associated with these rare tumors.

Given the rarity of extensive SGBCC lesions, there is no established consensus on treatment approaches. However, when feasible, surgery remains the treatment of choice, with wide excision recommended to achieve clear margins. For aggressive and large BCCs, excision margins of 13 mm or more are typically required, in contrast to the 4 mm margins often acceptable for smaller BCCs [13]. In cases of lymphatic spread, lymphadenectomy may be considered. Mohs surgery also plays a key role in ensuring complete excision, especially for tumors with aggressive characteristics. Surgical outcomes tend to be more favorable in patients with large exophytic lesions amenable to resection, with these patients generally exhibiting lower mortality compared to those who are not surgical candidates [1,14].

In cases where surgery is not possible, alternative treatments such as the hedgehog pathway inhibitor vismodegib have shown promise. Approved by the U.S. Food and Drug Administration in 2012 for locally advanced and metastatic BCCs, vismodegib has been used for tumor debulking and as an adjunct to surgical resection. Although response rates for metastatic and locally advanced BCC are relatively low, ranging from 30% to 43%, it remains a viable treatment option [1,15]. For surveillance, some experts recommend regular clinical follow-ups every three months, including yearly MRI scans, although patient compliance may present challenges. Treatment may often be multimodal, combining surgery, radiation therapy, and immunotherapy to improve outcomes and manage the aggressive nature of SGBCC [1,12].

CONCLUSION

This case highlights the destructive potential of super giant basal cell carcinoma (SGBCC) when diagnosis and treatment are delayed, emphasizing the critical importance of early detection and intervention to prevent extensive morbidity. The diagnostic challenges, including its ability to mimic other conditions such as inflammatory breast cancer and pyoderma gangrenosum, underscore the need for comprehensive clinical and dermoscopic evaluations. Our findings reinforce the necessity for a multidisciplinary approach and personalized treatment strategies to improve outcomes for these exceptionally rare and aggressive tumors.

REFERENCES

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